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A 36-year-old man used to request multiple repetitions of spoken words in order to understand what was said. Relatives and family members advised him to consult an ENT surgeon for hearing impairment. No organic cause of hearing impairment was found. He was then referred to a neurologist and a general physician for evaluation of neurological cause of his symptoms. Even after multiple consultations with the ENT, neurology and medicine specialists, no organic pathology to explain his symptoms could be detected. The patient lost considerable time and money because of delay in diagnosis caused by the atypical presentation to specialties other than Psychiatry. At last, the patient was referred to the Department of Psychiatry for further evaluation. The patient's chief complaints were difficulty in hearing, grasping or comprehending the components of conversations, interactions or clinical interviews at once. He had this problem for the last 6 months. During the interview, almost every question or the instruction had to be repeated slowly 2 to 3 times or more to make him understand. He was anxious and expressed regret repeatedly for the inconvenience caused to the doctor because of his disability. Initially the patient was put on mirtazapine 15 mg/day and clonazepam 0.75 mg/day, keeping in mind the possibility of anxiety disorder NOS (not otherwise specified). There was no significant improvement even after 4 weeks of therapy. The case was reviewed from the diagnosis point of view. Mental state examination of the patient revealed impaired attention and concentration with depressive and anxiety symptoms. Detailed evaluation of the patient revealed that his mind remained full of unnecessary thoughts. However, the patient could not express further details to enable definite diagnosis. The possibility of OCD, predominantly obsessive type, was kept on the basis of answers of leading questions, and he was put on fluoxetine 60 mg/day (titrated from 40 to 60 mg) and clonazepam 0.5 mg/day. There was drastic improvement in all the presenting symptoms, including his ability to grasp and comprehend conversation, along with improvement in anxiety symptoms and clarity in thinking over a period of 6 weeks. The patient was then able to report his pre-treatment problem and the level of improvement comprehensively. He accepted that he was unable to understand or express his own problem before treatment. According to him, there were thoughts keeping his mind busy all the time, to the extent that he was unable to think or communicate his problem effectively. According to him, initially he used to make frequent unsuccessful attempts to get rid of these excessive and unnecessary thoughts. According to the wife of the patient, after treatment the patient was able to hear clearly. The diagnosis could be confirmed as OCD on the basis of patient's spontaneous elaboration of symptoms and the level of improvement. |
A 53-year-old female patient presented to the outpatient department of E.N.T. at the Barasat Cancer Hospital, Kolkata, India, with complaints of a slowly growing large swelling in the left lower jaw for a duration of 14 months. There was slight difficulty in chewing and swallowing due to the size of the tumor. On inspection a firm smooth lobulated mass was seen arising from the lingual aspect of the left side of lower jaw of 5 cm by 2 cm in size extending from canine to the premolar teeth occupying the edentulous portion of left half of mandible and adjacent floor of mouth. The mass was painless and nontender on palpation. The teeth on the left half of mandible were loose and patient was unable to approximate the jaws completely; hence, he could not chew or swallow effectively.\nThe rest of oral cavity oropharynx and laryngopharynx were normal on examination. There were no palpable cervical lymph nodes. Other than diabetes patient had no significant systemic ailments. Other than elevated blood sugar the routine haemogram, serological studies, X-ray of chest, and ECG were within normal limits. An orthopantomogram of the jaw showed a lobulated swelling in the left side of floor of mouth. The hyperglycemia was controlled with insulin therapy on admission of the patient. A punch biopsy from the mass done elsewhere was reported as oncocytoma.\nThe patient was planned for surgery. By a left lip splitting cervical collar line incision a flap was elevated and tumor was exposed. The tumor was attached to the inner (lingual) aspect of the left half of mandible encroaching upon the edentulous part of the mandible. The tumor was excised using electrosurgical instrument along with a marginal mandibulectomy of the involved part of the mandible. The mucosal flaps were elevated from the floor of mouth and buccal mucosa was released to bury the mandibular defect. The wound was closed in layers over corrugated rubber drain. The patient was kept on broad spectrum antibiotics, anti-inflammatory agents, and insulin on a sliding scale along with antiseptic mouth washes. The postoperative period was uneventful. Patient recovered well and was discharged from hospital 10 days after the removal of sutures. |
A 56-year-old previously healthy female presented with spontaneous vertigo that had first appeared more than 2 weeks earlier. She had experienced left-sided facial palsy 2 months previously. A bedside examination showed spontaneous right-beating nystagmus that became stronger during rightward gaze or in darkness without visual fixation, and positive rightward head-impulse signs indicative of acute left vestibulopathy, in addition to preexisting complete left facial palsy. The initial MRIs revealed segmental enhancement in the left facial and vestibular nerves at the distal auditory canal (). The findings of serological and CSF evaluations for infectious and autoimmune disorders were normal.\nThe patient was diagnosed with an inflammatory disorder of uncertain origin that involved the left internal auditory canal. She initially took oral prednisone at 1 mg/kg for 7 days. The dosage was then rapidly reduced over the following 4 weeks due to excessive weight gain, formation of a moon face, nervousness, and stomach irritation. The spontaneous nystagmus disappeared, but she still suffered from fluctuating dizziness. Follow-up MRIs obtained 4 months after the onset showed stable lesions in the left auditory canal and newly developed contrast-enhanced lesions in the left olfactory groove and right high-cervical lymph node (). The findings of the second evaluations for autoimmune and infectious disorders were also normal, and a histopathological evaluation performed at the right neck lymph node revealed chronic granulomatous inflammation without evidence of tuberculosis, nontuberculous mycobacterial infection, or malignancy such as lymphoma ().\nThe patient was finally diagnosed with cranial IIP and started on azathioprine with short-term prednisolone. Her symptoms remained stable while taking azathioprine, and a second follow-up MRI obtained at 7 months after the onset showed mild improvement of lesions involving the left olfactory groove and internal auditory canal (). However, serum aspartate and alanine aminotransferases had increased sixfold, while other liver enzymes were also elevated. Hence, despite some clinical improvements, the patient discontinued azathioprine and then was started on tacrolimus after normalization of the liver enzymes. The third follow-up MRIs obtained 17 months after the onset showed further improvement of the previous lesions (). Her facial palsy and vertigo had also improved—though not completely—through treatment with tacrolimus alone. |
A 38-year-old woman complaining of bloody diarrhea and general fatigue was admitted to the emergency department and was then hospitalized. She stated that she was being treated for Ulcerative colitis for the past 9 months and her symptoms had started a year ago with generalized fatigue and weight loss. After 3 months she presented other symptoms including abdominal pain and rectal bleeding therefore, upper endoscopy and colonoscopy was carried out. When she was referred to our center, she had been under treatment for 3 months and her medical record which included upper gastrointestinal endoscopy and colonoscopy revealed crypt destructive colitis and chronic gastritis. With these findings, she was diagnosed with early UC and was under treatment with mesalazine (3 g daily) and pantoprazole (40 mg daily). In addition, her routine blood tests had revealed pancytopenia before. This had led the doctor to obtain a bone marrow biopsy which was inconclusive due to an unsatisfactory sample, and also to request rheumatologic laboratory tests which were reported negative (Figures , , ).\nThe patient showed symptoms of general fatigue and lethargy along with fever and night sweats. She also stated that she had involuntarily lost 14 kg in 3 months. Her family members also stated that their younger sibling also had a history of an unknown rheumatologic disease and was expired years ago. Her physical examinations showed bilateral pitting edema in both legs, oral ulcers. She also suffered from bloody diarrhea and hemoptysis. When she was admitted she was severely ill. Her lower limb forces were reduced (3/5) and she could barely stand or walk. Her neck examination showed enlarged lymph nodes on the back of her neck. However, her vital signs were stable. As the patient's clinical signs were suggestive for SLE or myositis we ordered prednisolone (1 mg/kg) before the laboratory results were ready. This significantly improved limb forces and hemoptysis.\nHer laboratory tests were as shown in the table (Tables , ). The significant findings were pancytopenia, mildly elevated liver enzymes, a high ESR, severely low reticulocyte count, high LDH and B12 levels. Her urine analysis revealed hematuria and bacteriuria. Her arterial blood gases were normal. As her hemoglobin levels were deemed too low, she received 1 unit of packed RBC. We also obtained another bone marrow biopsy which was not in favor of malignancies. Another notable and important finding is that her serum protein electrophoresis revealed an unusual abnormality which was increased gamma globulin levels while albumin levels were low (Table ). This presentation was suggestive of gammopathy.\nIn her following work up a spiral chest computed tomography (CT) scan without contrast was requested that revealed ground-glass opacities in the lower lobes of both lungs. This finding was more prominent in the left lung. This finding was also suggestive of pneumonia. Other remarkable findings were mosaic attenuation in the parenchyma of both lungs, air cyst (12 ×12 mm) in left upper lobe, bilateral mild pleural effusion, cardiomegaly, and mild pericardial effusion.\nAs the findings were compatible with pneumonia, antibiotic therapy was initiated and she received 4.5 g of piperacillin/tazobactam intravenously 3 times a day and 750 mg of levofloxacin daily orally.\nDue to swelling and edema in her lower limbs, she underwent venous color Doppler sonography and nothing remarkable was observed. The patient also underwent neck sonography and many reactive lymph nodes were found on both sides of the neck. The largest ones were (30 ×9.5 mm) in zone 2 of the right side and the other (23 ×7 mm) in zone 2 of the left side. Other sonographic evaluations revealed axillary lymph nodes as big as (31 ×15 mm) on the left and (25 ×17 mm) on the right, respectively.\nShe also underwent echocardiography to assess cardiac activity and the prominent finding was a decreased ejection fraction (EF) (45%). As her medical condition and the hematochezia had worsened 5mg of prednisolone 3 times a day and also a single unit of IVIG 60 mg was ordered.\nAs the clinical manifestations of the patient were consistent with SLE, a rheumatologic consultation was requested. Which suggested laboratory tests that turned out to be positive for anti-dsDNA and also her urine analysis manifested severe proteinuria. Meanwhile, she had already shown symptoms of polyserositis, mouth ulcers, and pancytopenia. With all the aforementioned findings and signs, SLE became a confirmed diagnosis for the patient. Thus, intensive therapy with rituximab induction therapy along with 500 mg of prednisolone pulse therapy for 3 days was started, we also used 200 mg of hydroxychloroquine daily simultaneously with a daily dose of 1.5 g of mycophenolic acid as a maintenance therapy.\nUnfortunately, though, 2 days after the initiation of therapy oxygen saturation levels suddenly dropped to 70% and the patient became dyspneic thus the patient was intubated. Unfortunately, the patient showed signs of hemoptysis which was suggestive of alveolar hemorrhage. She was intubated and 2 units of fresh frozen plasma (FFP) were transfused. An emergent chest X-RAY was obtained that showed diffused patchy infiltrates that resembled either pneumocystis pneumonia (PCP), alveolar hemorrhage, or pulmonary thromboembolism (PTE). Portable echocardiography showed an even more decreased EF (40%) along with mild right ventricle enlargement and moderate left ventricle dysfunction. thereafter a new course of antibiotic therapy started with intravenous (IV) cotrimoxazole, vancomycin, and cefepime. The patient received another 500 mg of methylprednisolone pulse therapy and 4 mg of dexamethasone IV, 3 times a day. However, the patient expired on the same day due to alveolar hemorrhage arrest. |
A 35-year-old female patient visited the department of Oral Medicine and Radiology with a chief complaint of swelling in the right side of the face for the past 6 months. Patient was apparently normal one year ago. Then she noticed swelling in the right side of the face for which she consulted a dentist and got two teeth extracted from the right lower back tooth region. She gave a history that the swelling was subsided. Again, after 6 months she started to notice the swelling started in the same region, which was initially smaller in size and gradually increased to the present size. There was no history of pain associated with the swelling.\nOn extra oral examination, diffuse swelling was present on right side of the face measuring about 3 × 5 cm extending anteroposteriorly from the corner of the lip to the angle of the mandible and superoinferiorly from corner of the lip to 2 cm below the inferior border of the mandible []. Skin over the swelling was stretched and shiny. No ulcerations or discharge was present. Facial asymmetry was present on the Right side. On palpation, the swelling was hard in consistency and non-tender. Skin over the swelling was pinchable. Mouth opening was not restricted. Lymph node examination revealed two submandibular glands, which were palpable, both measuring 1 cm in diameter, firm in consistency, tender, and freely movable on the right side.\nOn intraoral examination, diffuse swelling involving the right lower alveolus extending from mesial aspect of 45 to retromolar trigone. There was obliteration of the buccal vestibule. Swelling was present on the lingual aspect extending to 1 cm above the floor of the mouth. The surface over the posterior aspect was erythematous and covered by white slough area []. No pus discharge or bleeding was present. On palpation, the swelling was soft to hard in consistency. Buccal and lingual cortical plate expansion was present. Provisionally diagnosed as benign tumor of the right mandible. Investigations carried out were panoramic radiography [], computed tomography [], blood investigations, and excisional biopsy. Panoramic Radiograph revealed a multilocular lesion in the right body of the mandible extending from the distal aspect of 43 extending to the right ramus of the mandible. There was destruction of inferior border and posterior border of ramus. Inferior alveolar canal was not appreciated. There was absence of resorption of roots 44, 45, 46. Computed tomography axial slice revealed diffuse expansile lesion in right side of the mandible with soft tissue enlargement of the same side. There was involvement of right angle and ramus of mandible with buccal and cortical plate destruction. 3D CT revealed the involvement of the right body, angle, and ramus of the mandible with bicortical plate expansion. Excisional biopsy was done [] and specimen [] was sent for histopathological examination, which revealed granular cell ameloblastoma of the right mandible []. |
A 41-year-old man, who had no significant previous medical or family history, was thrown from the rear seat of a vehicle during an accident on the motorway. He was transferred to the emergency department of our hospital. Upon admission, he had an open airway, normal breathing with a respiratory rate of 16 breaths/min, was hemodynamically stable with a blood pressure of 123/79 mmHg, and a pulse rate of 88 beats/min. He was conscious and scored E3 for eye opening, V5 for verbal response, and M6 for motor response on the Glasgow Coma Scale. He had a left pneumothorax, a left, open, mid-shaft clavicle fracture accompanied by a 10 mm-sized laceration with numerous subcutaneous air bubbles trapped in the soft tissue on the lateral end of the clavicle, and left subclavian arterial injury (Gustilo Grade I) (\n). He had multiple lacerations of the forehead without abnormal findings in computed tomography of the head and neck. Both hands were warm with brisk capillary refill in the fingers. The radial and ulnar pulses in the left hand were palpable, but markedly weaker compared to those of the right hand. The blood pressure of the left arm was approximately half that of the right arm blood pressure. Despite no muscle weakness in the upper extremities, the patient had left hand numbness. The Injury Severity Score was 11. The patient was treated with urgent debridement and irrigation for the open clavicle fracture in the operating room followed by urgent angiography for the subclavian artery injury. Initial selective angiography of the left subclavian artery via the right common femoral artery revealed a segmental dissection of the distal subclavian artery with preserved blood flow to the left upper extremity (\n). Subsequent intravascular ultrasound via the left brachial artery revealed an intimal flap and a compressed true lumen by a thrombus of the pseudo lumen in the distal subclavian artery (length of the lesion, 3 cm). An 8 mm × 40 mm self-expanding nitinol stent (Smart Control, Cordis) was deployed. Adequate stent expansion and restoration of blood flow of the subclavian artery were confirmed (\n). After the endovascular stenting, the left radial and ulnar pulses were remarkably improved and the blood pressure difference between the left and right arm was significantly eliminated. Antithrombotic therapy to prevent stent thrombosis using intravenous heparin targeting aPTT of 2 times the control aPTT for 9 days was followed by an antiplatelet therapy using aspirin 100 mg plus cilostazol 200 mg daily for 12 months. On day 6, an open reduction and internal fixation of the clavicle fracture using a Kirschner wire were performed. The patient was discharged on day 22 and continued to be free of complications at the 2-month follow-up with stent patency determined using color duplex ultrasonography. |
A 13-year-old girl was admitted to hospital because of a urinary tract infection. Ultrasonography revealed a 5 cm round mass located in the head and body of the pancreas. Computed tomography (CT) confirmed diagnosis and was characteristic of Frantz tumor (Figures and ). The patient was referred for surgical treatment. The location of the tumor indicated that pancreaticoduodenectomy should be the procedure of choice but favorable anatomical conditions during the operation allowed central resection of the tumor. The head of the pancreas from the duodenal border to the tumor border accounted for more than 3 cm, and allowed for 1 cm safe margins in resection from both sides of the tumor. The proximal pancreatic stump was over 7 with interrupted nonabsorbable 2–0 monofilament suture (Prolene 2-0 Ethicon). The distal part of the body and tail of the pancreas were anastomosed with a Roux-en-Y loop (double layer cuff anastomosis, Figure ). Two closed suction drains were placed in the operative field. The early postoperative course was uncomplicated but on the 10th day after the operation active bleeding in the abdominal cavity was observed. Exploratory relaparotomy was performed and revealed active bleeding from a small arterial branch of the pancreatic artery in the connection between the Roux-en-Y loop and the tail of the pancreas. The bleeding was stopped with one monofilament stitch. In the postoperative course, pancreatic leakage was observed through the drains with amylase levels of more than 100,000 units and a loss of about 100 mL per day. The patient was managed with nothing by mouth and the leakage diminished during the course of a week, and stopped spontaneously 20 days after the 2nd operation. The serum level of amylase was below 500 units throughout the postoperative course. Pathological investigation confirmed epithelial solid papillary cystic neoplasm, and no adjuvant therapy was recommended. Control CT investigation did not reveal any relevant lesion, and the patient was discharged from hospital 30 days after the operation. The time of observation is 3 years. Informed consent was given by the patient for using her clinical data. |
A 60-year-old South Indian female presented with a swelling on the left zygomatic region of the face since a year with a history of the swelling initially appearing small in size which subsided. It later recurred a month before visiting the clinic. The swelling was associated with fever with no other associated history of epistaxis or headache.\nOn clinical examination, extraoral inspection revealed slight fullness in the left maxillary region. There was a firm, nontender mass palpable in the floor of left orbit just below the inferior orbital margin. The swelling measured about 4 cm in diameter with the surface appearing normal []. Visual acuity, visual field, and eyeball movements were normal. There was no regional lymphadenopathy. A small nasal polyp was noted on the left nasal cavity. Palpation revealed it to be tender and soft.\nThe intraoral inspection revealed her oral hygiene to be poor. Malaligned dentition with calculus debris and mobility was seen. There was an intraoral swelling seen at the palatal region measuring about 5 cm in diameter extending from 24 to maxillary tuberosity region. On palpation, it revealed to be tender and hard to firm [].\nOcclusal radiograph and orthopantomogram revealed the presence of minute radiopaque structures resembling calcifications in an ill-defined hazy radiopacity in the left maxillary sinus suggestive of the residues of the eroded bone [Figures , and ]. Cone-beam computed tomography (CBCT) revealed soft tissue opacity in the left maxillary sinus with the extension of the mass into the left nasal cavity almost invading the nasal septum along with prominence in the calcification seen in the center of the sinus cavity as well []. Bony erosions were seen involving the left floor of the orbit, nasal wall, and nasal septum. The sinus cavity appeared expanding as an expression of the pressure due to the growing mass [].\nThe patient was further sent to the Department of Oral Pathology for a biopsy which conferred to be schwannoma. The given hematoxylin and eosin section showed a spindle cell proliferation with some areas showing palisaded arrangement of the nuclei around a central acellular eosinophillic area. Other areas showed cellular, unorganized spindle cells arranged randomly in a loose myxomatous stroma. Focal areas of nuclear atypia were seen. The lesion was capsulated. Focal areas of dilated blood vessels were also seen. A biphasic pattern was seen with the presence of both Antoni Type A and Type B cells [] Necrotic areas and degenerative changes were not evident. Confirmation was carried out on performing immunohistochemical (IHC) staining using S-100 marker [].\nClinical differential diagnosis can be given as odontogenic tumors such as ameloblastoma but in our case there was no unerupted teeth or egg shell crackling, fibro-osseous lesions like fibrous dysplasia but it does not occur in older age groups, a soft tissue malignancy due to its enormous growth. Neurofibroma also originates from Schwann cells and must be considered as a differential during the histopathologic examination.\nOn being diagnosed with schwannoma, the patient underwent hemimaxillectomy by Modified Weber Fergusson approach (with Lynch extension). Initially, tarsorraphy sutures were given to close the patient's eyelids. The outline of the purposed approach was highlighted using a marker. The marking was drawn starting from the lower border of the left eye extending until 2 mm from the medial canthus, it was extended downward along the lateral border of the nose until the midpoint of the philtrum of the upper lip dividing it into two equal parts.\nIntraorally the incision was continued through the gingival margin and was connected with a horizontal incision at the depth of the labiobuccal vestibule extending back to the maxillary tuberosity. The incision was then given medially across the posterior end of the hard palate further turning to 90° anteriorly to the proximal side of the midline.\nExtraorally the incision was given deeper onto the bone following the markings right from the upper lip except for the lower eyelid area to preserve the orbicula ris oculi muscle. The incision extending around the nose was deepened to the nasal cavity. The cheek flap was reflected and the lesion was exposed. The gingival and palatal mucosa were elevated back to the midline. The palatal bone was divided near the midline using a saw blade and bur, the basal bone was separated from the frontal process of the maxilla with an osteotome. The orbicularis oculi muscle was retracted superiorly and the bone was cut extending across the maxilla just below the inferior orbital rim. The entire specimen by hemimaxillectomy was removed in toto severing the remaining attachments using a large curved scissor placing behind the maxilla. The excessive bleeding was controlled with electrocautery, and all the sharp bony projections were trimmed off.\nThe palatal flap was turned up to cover the medial bony margin, and the hollow space created postsurgery below the orbital floor and the maxillary region was covered with a packing made up of acrylic resin, wrapped with the skin graft taken from the patients shin area of the leg was used. Finally, a prefabricated obturator was placed to seal the defect and support the packing. The obturator was fixed to the overlying zygomatic bone. The extraoral reflected cheek flap was sutured to its normal anatomical site [Figures and ]. |
A 79-year-old female patient with hypertension presented with a compression fracture of the L5 vertebral body. The orthopedic surgeons performed PKP. Three weeks later, the patient developed chest pain with chest tightness. An electrocardiogram showed signs of myocardial ischemia. Chest tightness and tachycardia were relieved after the administration of oral antithrombotic clopidogrel; however, the patient still felt occasional chest pain. No heart murmur was observed in the physical examination, the chest x-rays suggested only an enlargement of the lung texture, and the laboratory tests showed abnormally elevated levels of myocardial enzymes; therefore, we suspected that the patient might have had coronary atherosclerotic heart disease. To determine the status of the patient's coronary artery, a coronary computed tomography angiography (CCTA) was conducted. The CCTA results indicated a high-density shadow extending from the bottom of the heart to the anterior chest wall and multiple areas of coronary stenosis. A computed tomography (CT) examination was conducted and the results revealed a mottled and striated high-density shadow in the right lower lung and a striated high-density shadow at the base of the heart (Fig. A). Because of the recent surgery, the possibility that this foreign body was bone cement was considered; consequently, the patient was immediately transferred to the department of cardiovascular surgery. An echocardiogram revealed a left ventricular ejection fraction of 60%, a nondilated atria, ventricles with normal systolic function, and a strong echo ∼6.8 cm long in the right ventricle, which penetrated through the tricuspid valve into the right ventricle. This type of injury leads to severe tricuspid insufficiency, with a large number of reflux signals in the tricuspid orifice and a small volume of pericardial effusion in the pericardial cavity. The patient underwent a coronary angiography and emergency surgery in the digital subtraction angiography (DSA) operating room on the same day. The coronary angiography suggested that there were multiple areas of coronary stenosis. A standard median sternotomy was performed. A cardiopulmonary bypass was established with the ascending aorta and bicaval cannulations. St. Thomas II solution was administered antegrade to induce cardioplegia following aortic cross clamping. The tricuspid valve was exposed through an oblique right atrial incision. Valve anatomy and regurgitation mechanisms were evaluated using a saline injection test. The cardiopulmonary bypass time was 86 minutes. During surgery, ∼100 mL bloody pericardial effusion was observed. The foreign body formed a needle-shaped foreign body that measured ∼8.0 × 0.3 cm. The majority of the foreign body was located in the right ventricle, piercing the posterior leaflet of the tricuspid valve and resulting in severe tricuspid regurgitation. The foreign body also pierced the anterior wall of the right ventricle and protruded from the apex cordis by ∼1.5 cm. The chordae of anterior leaflet and septal leaflet ruptured, and the ventricular surface of the septum had the appearance of jelly-like fine-grained neoplasms (Fig. B–D). The foreign body was removed and the tricuspid valve was repaired. Two 4–0 Gore-Tex sutures were used as artificial chordae to reconstruct the subvalvular support system and coronary artery bypass grafting surgery was performed to treat the coronary stenosis. The embolism in the right lower lung was not removed because there were no obvious respiratory symptoms. According to the patient's surgical history, the characteristics of the foreign body, and the same density of shadows in the right lung at CT image, we speculate that this foreign body was bone cement. The postoperative course was uneventful and the patient was discharged on the 13th day after surgery. Echocardiography before discharge showed mild reflux in the tricuspid valve and no effusion in the pericardial cavity. An echocardiograph of the patient after 49 months indicated that tricuspid regurgitation was not aggravated. The patient was given a Class II functional classification according to the New York Heart Association. |
A 52-year-old man presented with upper abdominal pain that had lasted for 1 week. MRI and CT suggested the presence of a gallbladder stone and a cystic tumor in the dorsal pancreas with a dilated pancreatic duct (Fig. ). Results of laboratory tests were normal. Cholelithiasis and pancreatic head cystadenoma were diagnosed, and cholecystectomy and head dorsal pancreatectomy were suggested for treatment.\nThe procedures used to expose the pancreatic head were the same as those described in the previous case. Palpation and intraoperative ultrasound examination were performed to confirm the presence of a cystic mass in the dorsal segment of the pancreatic head. The EFP appeared as a faint line of loose fibrous connective tissue in the inferior pancreatic head (Fig. A). Dissection along the EFP was performed easily, with no major vessels to avoid, and the ventral and dorsal segments were divided (Fig. B). The main pancreatic duct was identified; this duct ran across the EFP from the dorsal segment to the ventral segment (Fig. B). The duct was ligated and cut off near the dorsal segment. Cholecystectomy was performed, and a catheter was inserted into the CBD via the cystic duct to help identify and preserve the intrapancreatic CBD. The CBD was located in the ventral segment, with its anterior surface constituting the right portion of the interface between the ventral segment and the dorsal segment. Dissection of the dorsal parenchyma was performed along the anterior surface of the intrapancreatic CBD.\nThe anterior superior pancreaticoduodenal artery was identified near the upper edge of the pancreatic head, and the collateral branches to the pancreas were carefully ligated. To insure the maintenance of blood supply to the duodenum and the remaining ventral pancreas, the posterior superior pancreaticoduodenal artery was preserved. The pancreatic neck was dissected and transected, and the dilated distal main pancreatic duct was carefully isolated and intubated (Fig. C). The head dorsal segment was totally divided and removed (Fig. D). An intraoperative pathological examination confirmed the diagnosis of serous papillary cystadenoma with negative margins (Fig. E). An end-to-side, duct-to-mucosa pancreaticojejunostomy was created using the Roux-en-Y method (Fig. F).\nThe patient experienced a grade B pancreatic fistula, which healed 25 days after conservative treatment. After 24 months of follow-up, there was no evidence of tumor recurrence.\nOnly 15 cases of anatomical segmentectomy of the pancreatic head along the EFP have previously been reported in the literature (Table ).[ |
A 55-year-old female patient from Southeast Ethiopia, presented to Goba referral hospital with complaints of right flank pain and swelling on the right lumbar region. The pain was a dull ache and not associated with urinary complaints like dysuria, frequency, urgency or hematuria. She had no cough and chest pain.\nThe patient reported that she had been visiting more than three hospitals with complaints of right flank pain for the last eight years and had been taking an ultrasound, magnetic resonance imaging, renal function test, and urine analysis. However, the patient reported that the results of these investigations were normal, and she was only treated with different types of anti-pain medications. Four years later, the patient noticed that swelling on the right lumbar region, and the swelling had increased gradually and was associated with pain. She had no history of flank trauma, surgery, chronic constipation, and other abdominal infections. In addition, the patient had no history of chronic cough, difficulty of urination, and history of diabetes mellitus.\nOn admission, the patient was clinically active and had normal vital signs. On abdominal examination, there was round protruding swelling immediately beneath the 12th rib of the right upper flank region (). The swelling was palpable and bowel sound was heard in the region. The swelling was easily reducible and protruded when the patient was coughing or straining. The opposite side lumbar region was normal, and chest examination was clear and normal. The hematological and urine analysis laboratory findings were normal. The ultrasound picture revealed the bowel contents within the hernial sac. Otherwise, all visceral organs were normal.\nWith the diagnosis of primary acquired superior lumbar hernia, the patient underwent open surgery. The open approach was designed due to the absence of laparoscopy services in the hospital. The defect was reduced back and repaired using a retro-muscular or sublay prolene mesh (15 cm by 7.5 cm) as this can maintain the maximum overlap of healthy tissue with the implanted mesh material. The patient was discharged at postoperative day four and followed up for four months. The patient had no recurrence of swelling at four months of follow-up. |
A 60-year-old male was referred to our department, complaining about gradually worsening hoarseness, during the last 8 month period. Occasional dysphagia and foreign-body sensation were also reported upon referral. The patient was a heavy smoker for more than 20 years, reporting an average of 20 cigarettes per day. Alcohol was also a factor, and although no real alcohol abuse or indulgence was noted, the patient was a rather frequent user.\nMedical history only revealed arterial hypertension under treatment with beta blockers. Haematological and biochemical tests did not show any significant abnormalities.\nPhysical examination included a full head and neck examination, complemented with flexible fiberoptic laryngoscopy. Typical ear, nose and throat examination did not reveal any abnormal findings and neck palpation was negative. However, fiberoptic laryngoscopy revealed a lesion affecting both vocal cords and anterior commissure, while vocal cord mobility appeared impaired. On these grounds, a cervicothoracic and upper abdomen computed tomography (CT) scan with intravenous gadolinium was decided and the patient was scheduled for direct microlaryngoscopy and biopsy of the lesion under general anaesthesia.\nImaging confirmed the laryngeal lesion, yet it also indicated a second lesion about 2 cm below the inferior end of the primary one, arising somewhere between the first and second tracheal ring. Intermediate tissue appeared grossly normal (). No signs of enlarged cervical lymph nodes were noted and laryngeal cartilages showed no abnormal findings.\nOn the other hand, histopathological examination after biopsy of the lesion under general anaesthesia confirmed the diagnosis of squamous cell carcinoma. The lesion was carefully mapped and proved to be a glottic carcinoma affecting the anterior commissure and appearing in strong correlation with the thyroid cartilage. The lesion infiltrated the left and the first tertile of the right vocal cord. No subglottic extension was noted. In this context, the patient was informed and consent for radical surgical therapy was obtained.\nThe patient underwent total laryngectomy and wide excision of the trachea which included the second tumour within safe limits (). The procedure was complimented with left thyroid lobectomy and bilateral selective neck dissection (Robin’s levels II–IV). Paratracheal lymph nodes (Robin’s level VI) were also carefully dissected. The overall postoperative course was uneventful. The patient was discharged from our department on day 16 with very good swallow function and was decannulated after 1 week. Surgical resection was followed by postoperative radiation therapy (6400 cGy/32 fraction).\nThe final pathological report was of crucial importance in our case. First of all, the surgical margins of resection were found to be free of disease. Second, histological sections from the tumour of the glottis showed the characteristic morphology of squamous cell carcinoma. Cancer cells were large in size and polygonal in shape with eosinophilic cytoplasm and nuclei with moderate variation in size and shape. There were a moderate number of mitoses and keratinisation could be focally observed. Cancer cells showed an infiltrative pattern consisting mainly of nests and trabeculae that invaded the vocalis muscle in both the vocal cords. The perichondrium of thyroid cartilage was focally invaded by cancer cells. Histological sections from the tumour of the trachea showed morphological features identical to those of the tumour of the glottis. An upward infiltrating pattern could be noticed. Moreover, a comparative immunohistochemical study of the two tumours showed strong positivity of cancer cells in stains for keratins AE1/AE3 and 34βΕ12 and moderate positivity in stains for CK5/6, CK8/18 and epithelial membrane antigen. Immunohistochemistry for D2-40 antigen (podoplanin) illustrated the positivity of the lymphatic endothelium. Immunohistochemical stains for other vascular endothelia (CD31 and CD34 antigens) were also performed, and were negative. In the region between the two tumours, many lymphatics containing neoplastic emboli could be observed (). Finally, two tumour-infiltrated lymph nodes (the larger being of 1.2 cm diameter) with extracapsular spread were found in the left neck dissection specimen. A pT4a(m)N2b stage, according to eighth edition TNM staging, was established. |
A 46-year-old lady was referred for investigation of a change in bowel habit. A computerised tomography (CT) colonography was performed and excluded any colonic pathology but did identify that the common bile duct was mildly dilated with a diameter of 8 mm, although no clear cause was found. Some reactive celiac lymph nodes were also seen and so the patient underwent an endoscopic ultrasound (EUS) scan to exclude a malignancy. The latter showed sludge and stones within the common bile duct and gallbladder. A subsequent endoscopic retrograde cholangiopancreatography-(ERCP-) guided sphincterotomy and balloon trawl of the common bile duct were performed leaving the common bile duct clear. Given the previous presence of gallstones within the common bile duct, it was decided to proceed to elective laparoscopic cholecystectomy. Laboratory investigations on the day before surgery revealed normal liver biochemistry (alkaline phosphatase 120 IU/L, bilirubin 3 umol/L, and alanine aminotransferase 23 IU/L).\nThe patient's medical history was remarkable for excision of an intracranial epidermoid cyst in 1993. The patient developed nonobstructive hydrocephalus following this procedure and required the insertion of an LP shunt. The shunt ran from the subarachnoid space at the level of fourth and fifth lumbar vertebrae, around the right flank, through the right rectus abdominis muscle and peritoneum and the tip lay within the peritoneal cavity in the pelvis. In addition to the hydrocephalus, the patient suffered from chronic chemical meningitis and headaches, which were managed with an occipital nerve stimulator and oral analgesia. The generator for the occipital nerve stimulator lay within the subcutaneous tissue in the right paraumbilical region and the wires ran cranially in the subcutaneous plane from this generator (). The patient otherwise had a history of well-controlled asthma, depression, gastroesophageal reflux disease, and coeliac disease.\nThe patient was admitted for an elective laparoscopic cholecystectomy under general anaesthesia. A number of modifications were made to the standard procedure for a laparoscopic cholecystectomy. Firstly, port placement was altered. A 10 mm port was created through the right rectus muscle 4 cm below and lateral to the umbilicus to avoid the occipital nerve stimulator generator. A Hasson's approach was used to place this port and this was then used to insufflate the peritoneal cavity. A further 10 mm port was placed in the midline within the epigastric region under direct vision. The light from the camera within the abdomen was used to identify the wire running cranially from the occipital nerve stimulator generator and thus ensure the epigastric port was placed safely. Two 5 mm ports were placed in the right side of the abdomen under direct vision to guide port placement around the LP shunt. These were sited within the right upper quadrant and the lateral aspect of the right abdomen at the level of the umbilicus. Secondly, the pneumoperitoneum pressure was set to 7 mmHg to minimise the risk of retrograde flow of gas along the LP shunt, carbon dioxide absorption into the blood, and change in venous pressure. This intervention was aimed at limiting any alteration in intracranial pressure during the procedure. Routine anaesthetic monitoring was used and no alterations were made to patient positioning with reverse Trendelenburg with the left tilt used during the procedure.\nThe patient tolerated the procedure well and there were no intraoperative complications. Postoperatively, the patient remained neurologically intact and there was no change in the frequency or severity of her headaches or other symptoms of raised intracranial pressure. The patient was discharged the day following the procedure. |
A 31-year-old male patient presented to our hospital on December 31, 2018 due to systemic erythema, papule and psoriasis with no obvious causes for 14 years, together with tumor mass in the tibial side of left lower limb for 6 months. For the erythema, papule and psoriasis, no systemic treatment was given except intermittent administration of TCM that may contain the arsenic. Recurrence was frequently reported by the patient, and the injury showed gradual progression. About 6 months ago, the patient showed a tumor mass in a pink color with no causes in the tibial side of left lower limb. The mass showed a diameter of about 1 cm at first, but then was in a size of 5.5 cm × 5.5 cm rapidly. Besides, ulcer, exudation, aberrant smell, and obvious pain were reported by the patient in the lesion sites. The urination and defecation were normal. He showed no obvious loss in the body weight. Each patient signed the informed consent. The study protocols were approved by the Ethical Committee of The Second Hospital of Jilin University.\nOn physical examination, there were no obvious abnormalities in the systemic examination. There was no swelling in the bilateral inguinal and popliteal lymph nodes. MRI indicated slightly long T1 and slightly long T2 signals in an irregular profile in the soft tissues at the inside of right leg. Local parts showed protrusion to the skin. The adjacent soft tissues presented long T2 signals in a patchy form. There were no aberrant signals in the right tibiofibula. Diffused papule and plaques in a dark red color were observed in the facial and cervical parts, trunk and limbs. The skin tissues in these parts were covered by massive scale in a silver color, especially the lower limbs. The findings for the phenomenon of membrane and dotty hemorrhage, and punctate hemorrhage were all positive. Multiple small papule (0.5 cm) in a jasmine color was observed in bilateral palms and the dorsal skin of the hand. The texture of the papule was hard, and the boundary was clear. No symptoms were felt by the patient at fist. The boundary between the mass in the left leg and the adjacent tissues was not clear, and the movement of the mass was poor. There was a black plaque with a width of 1.5 cm that wrapped the base of the peripheral mass (Fig. ).\nFor the laboratory test, the urine arsenic was in a concentration of 24.67 μg/L (normal range: <300 μg/L), while the arsenic content in hair was 0.331384 μg/g (normal range: <0.06 μg/g). There were no aberrant changes in the other tests. Pathological analysis for the mass in the left lower limb indicated squamous carcinoma (Fig. ). Pathological analysis for the black plaque in the base indicated pseudoepitheliomatous hyperplasia combined with hyperkeratosis, as well as slightly chronic inflammatory cell infiltrate in the epithelium (Fig. ). On this basis, the patient was diagnosed with psoriasis, highly differentiated squamous carcinoma in the left lower limb, and keratoacanthoma in left lower limb, as well as araenical keratosis.\nThe treatment regimen involved administration of Acitretin (30 mg/day), application of humectant and momestasone furoate, and anti-infection therapy, lasting for 3 weeks. The skin lesions were stable with no progression. Then the dose of Acitretin was reduced to 10 mg per day. On this basis, extensive resection and free skin grafting were performed under general anesthesia. Postoperative pathological analysis indicated no cancer cells at the margin when extending the cutting margin to a diameter of about 1.5 cm. Therefore, the intraoperative resection scale was at a position that was about 1.5 cm to the tumor margin. The deep fascia was resected, and the defect area showed a size of 10 cm × 10 cm. The full-thickness skin graft with the same sized psoriasis plaque was obtained from the homolateral abdomen, and then the donor site was directly sutured afterwards. The wound surface was covered by full-thickness skin graft, followed by packaged suturing through intermittent knot and compression. After surgery, the affected limb was elevated to 45 degree, together with anti-infection, improvement of microcirculation and regulation of epithelium (Fig. ). On postoperative day 4, the surgical region showed isomorphic reaction of the psoriasis. The peripheral skin injury showed progression, which may be related to the surgical stress. Therefore, the dose of Acitretin was elevated to 30 mg per day. On day 14, the bandage was removed, and the transplanted graft was alive with few vesication and punctiform ulcer. The patient was followed up for 8 months with satisfactory outcome (Fig. ). The color and texture of the free skin graft were similar with the recipient site. Additionally, the psoriasis showed attenuation and even elimination. |
A 57-year-old Caucasian woman presented for a total body skin check in November 2016. A melanoma in situ on her left proximal arm had been diagnosed five months earlier; an excision of the site had been performed two months ago. There was no evidence of recurrence and no palpable axillary lymph nodes.\nHer past medical history was remarkable for right triple negative (estrogen receptor, progesterone receptor, and HER2) invasive ductal carcinoma of the breast diagnosed in June 2015. She received 12 weekly doses of paclitaxel followed by doxorubicin/cyclophosphamide every two weeks for four cycles. In December 2015 she had a right lumpectomy and sentinel lymph node biopsy; none of the three nodes were positive for cancer.\nShe completed radiation therapy in March 2016. Adjuvant capecitabine was recommended; in May 2016 she began oral therapy at a dose of 1500 mg twice daily; within one week, the dose was increased to 1650 mg twice daily for 14 days on and seven days off for eight cycles. She developed hand-foot syndrome during the initial cycle. This persisted during her treatment, but never greater than grade 1 in severity; neither discontinuation nor dose reduction of capecitabine was necessary.\nFollowing the initial cycle of therapy, she became aware of a loss of fingerprint quality. She was no longer able to gain entrance into her fitness center that required index finger scanning for identification to access the facility. In addition, prior to starting capecitabine, she was able to open her smartphone by pressing her index finger on the screen; after her first cycle of capecitabine, the personal electronic device could not confirm her identity based on evaluation of her fingerprint and she has to enter a four-digit code to be able to use her telephone.\nExamination of her hands in November 2016, just prior to her completing the final cycle of capecitabine, showed erythema of the palmar surface of her fingertips (Figures -) and thumbs (Figures -). A closer examination of her fingertips showed occasional distal fissures and an absence of the dermatoglyphics (Figures -). A closer examination of her thumbs showed scaling, random fissures and focal preservation of only some of the fingerprint ridges (Figures -). However, the loss of her fingerprints on her digits was severe with absent functional quality of her finger dermatoglyphics. |
We present the case of a 77-year-old ambulatory man with hypertension, sarcoidosis, complete atrioventricular block status post-pacemaker implantation, chronic kidney disease due to FSGS, and right facial nerve paralysis, who presented with sporadic gait and right face numbness. He was diagnosed with sarcoidosis by biopsy of a tumor in front of the right tibia 14 years before presentation. Since the tumor and abdominal lymphadenopathy were the only manifestation of sarcoidosis and no other signs of organ involvement were present, he received no immunosuppressive treatment. The abdominal lymphadenopathy had been stable over time. Nine years before presentation, he was referred to our nephrology clinic to determine the cause of chronic kidney disease. His serum creatinine level was 1.2 mg/dL and he had proteinuria of 0.4 g per day. Hematuria was not present. Renal biopsy revealed six globally sclerotic glomeruli among all 34 glomeruli (18%) and some residual glomeruli with segmental sclerosing lesions, but no involvement of sarcoidosis. He was diagnosed with primary FSGS. Since the proteinuria was mild, he did not receive immunosuppressive treatment.\nOne year after that, the patient experienced palpitations and was diagnosed with complete atrioventricular block. Coronary angiography showed no significant stenosis of the coronary arteries, and he underwent pacemaker implantation. Whether sarcoidosis contributed to the complete atrioventricular block was unclear. The abdominal lymphadenopathy and the dyskinesia of the ventricular septum were stable and did not progress over time.\nThe patient was stable for eight years, until when he started to suffer from sporadic gait and right face numbness that occurred and resolved within a day every few weeks. Three months later, the symptoms recurred along with sudden dysarthria and left limbs weakness. Physical findings were notable for pronator drift on the left side. Perfusion computed tomography (CT) with iodinated contrast and CT angiography revealed no ischemic lesions or occlusion of major cerebral arteries. The symptoms disappeared three hours after the onset. A transient ischemic attack (TIA) was suspected, and he was admitted to the stroke unit. Ultrasonography revealed no stenosis of the internal carotid arteries, and transesophageal echocardiogram showed no abnormalities of the atrial septum. His pacemaker detected paroxysmal atrial fibrillation, which was presumed to be the etiology of the TIA. Thus, edoxaban 30 mg per day was started and he was discharged after one week of hospitalization.\nOne month after his discharge, his left leg started to swell and his gait worsened. Urinary protein excretion was 0.6 g per day, serum creatinine was at the baseline level of 1.6 mg/dL, and serum albumin level was 3.8 g/dL. Although no coagulopathy was found, ultrasonography revealed left femoral vein thrombosis that was 41 mm long. Edoxaban was stopped, and heparin was administered intravenously for two weeks. Low mobility due to his gait was presumed to be the cause of development of deep vein thrombosis (DVT). The patient was switched to warfarin and was discharged, but the left leg edema persisted. Three months later, he developed complications of urinary retention and constipation.\nFour months after discharge, the patient presented to the emergency department with sudden left leg pain and inability to walk. The entire left lower limb was slightly pale and had slow pitting edema. The left dorsal artery was not palpable, and the left femoral artery was barely palpable. Contrast CT revealed occlusion of the left femoral and superficial femoral arteries together with the known DVT in the left femoral vein (Fig. , ). Emergency thrombectomy for acute arterial occlusion was performed and the leg perfusion resumed. The emboli (maximum of 23 mm in diameter) were sent for pathological examination. The patient was admitted to the hospital and started on heparin infusion in place of oral warfarin. The history of recent TIA implied hypercoagulable state, but again no coagulopathy was found. While malignancy screening was being planned, the pathology of the arterial emboli revealed an unusual and surprising finding: the surface of the thrombi was filled with large atypical lymphoid cells (Fig. ) and was covering the necrotic interior of the thrombi. Immunohistochemical analysis showed that the tumor cells on the surface and the necrotic interior of the thrombi were positive for CD20 and CD79a but negative for CD3 (Fig. , ), which is characteristic of B cells. Leukocytosis was absent (white blood cell, 4,000/μL; segmented neutrophil, 55%; lymphocyte, 34%; monocyte, 9%; eosinophil, 2%). Serum soluble interleukin-2 receptor level was 1,548 U/mL (normal, 122–496 U/mL); lactate dehydrogenase (LDH) level, 808 U/L (normal, 120–245 U/L); LDH-2 fraction, 39% (normal, 28–35%), and LDH-3 fraction, 32% (normal, 21–27%). These findings were consistent with large B-cell lymphoma with intravascular proliferation, but the etiology of the aortic thrombi was unclear.\nThe hematology consultation team considered that the patient needed further biopsy to determine the etiology. Bone marrow biopsy showed normocellular marrow with normal maturation, but with infiltration of CD79a-positive large atypical lymphoid cells within the small vessels (Fig. , ). Although no lymphadenopathy was detected on palpation, CT scan showed swollen bilateral axillary and inguinal lymph nodes, which were up to 30 mm in diameter. While surgical biopsy of the right axillary lymph node and random skin biopsy were planned for diagnosis, the patient developed a complication of sepsis presumably due to pyelonephritis on hospital day nine. Piperacillin/tazobactam and vancomycin were started. Because partial thromboplastin time was prolonged, biopsies were withheld. Although white blood cell and neutrophil counts were improving, the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. The patient had a do-not-resuscitate order. His family agreed to an autopsy. |
A 3-year-old male child had been born at term after a normal delivery. At the age of 1 year, he underwent surgery for lumbosacral lipomeningocele. At that time, he presented with congenital swelling in the lumbosacral region without any neurological deficit. On examination, there was 3 cm × 3 cm size eccentric swelling (more on the right side) present in the lumbosacral region with intact overlying skin without any dermal sinus. On magnetic resonance imaging (MRI) of the lumbar spine (LS), there was evidence of dorsal spinal and subcutaneous lipoma with L2–L3 vertebral posterior arch defect. The Patient underwent excision of the spinal and subcutaneous lipoma [Figure –]. Postoperative period was uneventful, and the patient was discharged on postoperative day 7 without any neurological deficit.\nAfter approximately 2 years of the primary surgery patient now presented with history of fever for last 1 month and lower limb weakness and seizures for last 15 days. The patient was asymptomatic in intervening period. On admission, the patient was conscious but drowsy; there was Grade 0 power in both lower limbs with the presence of neck rigidity. MRI of the LS was suggestive of peripheral contrast enhancing lesion extending from D10 to L5 with diffuse expansion of cord suggestive of intramedullary abscess [Figure and ] With impression of intramedullary abscess, the patient was taken for surgery; exploration of previous incision with D12 and L1 laminectomy was done. Limited midline myelotomy was done with drainage of pus, and canal was irrigated with saline.\nPostoperatively, the patient was given broad-spectrum intravenous antibiotics for 2 weeks followed by 6 weeks of oral antibiotics. Culture sensitivity of pus was sterile. The patient was discharged on postoperative day 10 with improvement in consciousness of the patient with slight improvement in power of the right lower limbs (up to Grade 1) and no improvement in the left lower limb. At 6 weeks follow-up, the patient was doing well with no significant improvement in power in comparison to immediate postoperative period. |
A 79-year-old man with a history of controlled hypertension had been in a restaurant, where he had got into an altercation with another customer. He had then been hit in the chest and fell to the ground. Although he could not recall details of the situation probably due to mild alcohol intoxication, according to an eyewitness in a restaurant, the hit was a single strong one in the center of his chest. He started feeling nausea 30 min later and requested emergency services. He was brought to the emergency department of another hospital complaining of nausea. On arrival, he was alert and oriented with almost-normal vital signs except for a relatively low blood pressure (92/68 mmHg). He denied chest or back pain, or any other accompanying symptoms. No obvious wounds were evident on physical examination. Laboratory data showed mild elevation of D-dimer (2.7 µg/mL), but other parameters were almost within normal limits. Despite stable vital signs and no signs of chest injury, the physician decided to conduct computed tomography (CT) of the head and chest without contrast, based on the history of chest trauma and the unexplained symptom of nausea. No abnormalities were seen on head CT, but massive pericardial effusion was demonstrated on CT of the chest and dissection was suspected in the ascending aorta (). Aortic dissection was considered as a cause of pericardial effusion. The patient was transferred to our institute for further evaluation. Contrast-enhanced CT revealed an aortic dissection localized at the ascending aorta with massive pericardial effusion (). A three-dimensional (3D) reconstruction of preoperative CT demonstrated dissected and dilated ascending aorta with a maximal diameter of 44 mm (). Given these findings, an emergent operation was performed. After opening the pericardium, massive hemorrhagic pericardial effusion was evacuated and hematoma was revealed on the pulmonary artery side of the ascending aorta (). There was no obvious laceration on the adventitia; however, it can be contained rupture into aortopulmonary window in view of massive hemorrhagic pericardial effusion. Cardiopulmonary bypass (CPB) was initiated with an arterial cannula inserted into the undamaged right side of the ascending aorta under transesophageal echocardiography guidance according to CT finding of intact arch vessels and a venous cannula via the right atrium. In addition to an arterial cannula in the ascending aorta, one more arterial cannula was inserted into the left common femoral artery for flushing out of debris. Moderate hypothermia (bladder temperature, 28°C) was induced and the ascending aorta was opened without applying an aortic clamp. Retrograde cardioplegia was used for myocardial protection throughout the operation. For cerebral protection during circulatory arrest, antegrade cerebral perfusion was selected. Intimal tears were present on the noncoronary sinus of Valsalva and the distal part of ascending aorta located at the lesser curvature, and the ostia of the coronary arteries were intact (). All dissection sites were excluded and ascending aortic replacement with a single-branched Dacron® graft (J Graft 28/9 mm; Japan Lifeline, Tokyo, Japan) was performed. At first, distal anastomosis was performed under lower body circulatory arrest, followed by proximal anastomosis with lower body circulation using the side branch of graft connected to an arterial circuit. Weaning off CPB was uneventful and the chest was closed without difficulties.\nThe patient was transferred to the intensive care unit under stable condition. He was extubated on the day of operation and was transferred to the general ward on postoperative day (POD) 5. Postoperative contrast-enhanced CT revealed no abnormalities at sites of anastomosis, with no residual dissection. Pathological findings of the mid-part of ascending aortic wall showed hemorrhage and fibrosis in the adventitia, whereas no abnormality was seen in the media (). The postoperative course showed no complications, and the patient was discharged home on POD 24 after setting up in-home nursing care services. |
A 50-year-old woman with no coronary artery disease risk factors had suffered from transient chest pain, dyspnoea, and paroxysmal neck swelling irrespective of emotional stress for ∼15 years (). Since the attacks often developed after meals, especially at full stomach, she took care not to eat too much. At the age of 44, she was admitted to the emergency department for the first time due to acute pulmonary oedema following the aforementioned attacks. Urgent cardiac catheterization showed no significant coronary stenosis and typical apical LV ballooning (), and finally she was diagnosed as having TTS. Later, at the age of 45 and 49, she developed the same clinical disorder. Interestingly, the LV wall motion abnormality was localized to the basal region at the second admission () and diffusely at the last time (no images available). Moreover, during the second admission, acetylcholine (ACh) provocation testing for coronary spasm was performed with negative results. Although local LV asynergy disappeared spontaneously in each hospitalization, she had frequently suffered from the same attacks after meals and was referred to our hospital for further investigation. She did not have any oral medication during the TTS episodes. On admission, she had no subjective symptoms or objective signs in electrocardiogram (ECG) or echocardiographic examinations. However, abdominal ultrasonography for screening purposes detected a right adrenal mass of 35 mm × 35 mm in size and scintigraphy imaging showed high uptake of M-iodobenzylguanidine in the tumour (). Furthermore, urinary catecholamine levels (especially normetanephrine) were markedly elevated (). Since pheochromocytoma was highly suspected from these data, we paid attention not to perform any contrast studies that could cause adrenal crisis. This tumour was urgently removed surgically and histological diagnosis of norepinephrine-secreting pheochromocytoma was made (). Ten days after the operation, when urinary catecholamine levels were normalized (), we performed cardiac catheterization and ACh provocation testing. As shown in , intracoronary administration of ACh (20, 50, and 100 μg) into the left coronary artery (LCA) and that of ACh (25 and 50 μg) into the right coronary artery induced epicardial coronary spasm accompanied by chest pain and ischaemic ECG changes. Chest pain, ischaemic ECG changes, and myocardial lactate production were already noted at the timing of administration of 20 μg of ACh into the LCA before the occurrence of epicardial coronary spasm at 100 μg, indicating that microvascular spasm (MVS) preceded epicardial coronary spasm. Thus, we diagnosed that she had both epicardial coronary spasm and MVS, and initiated drug therapy with a calcium antagonist (benidipine 8 mg/day). After discharge from our hospital, she experienced no episode of postprandial attacks and her urinary catecholamine levels remained low, indicating that she was free of catecholamine surges from pheochromocytoma (). She again underwent a scheduled follow-up ACh provocation testing at 18 months after the operation. Calcium antagonist was discontinued 72 h before the provocation testing. Interestingly, epicardial coronary spasm was no longer induced, whereas MVS persisted (). |
We report on a case of a 16-year old male patient who was diagnosed with DF of the right upper thorax in 2011 (Fig. ). The primary approach consisted of partial tumor resection for functional preservation. Systemic therapy with MTX/VBL was started after completion of surgery in order to maintain tumor control. However, MTX-associated nausea and fatigue led to discontinuation of MTX after 6 months of treatment. Instead, sulfonylurea had been added to VBL, but again, nausea limited the applicability of this combination and treatment was provided as single agent sulfonylurea.\nTwo years after the initial resection, a symptomatic tumor recurrence was detected while receiving medical treatment. Local recurrence involved the right brachial plexus, which was incompletely resected for the purpose of function preservation.\nSystemic therapy was reconvened with MTX and vinorelbine, which achieved symptomatic improvement of pain with disease stabilization as best response on MRI. However, the treatment was not sustainable because of grade 1 nausea and fatigue and led to treatment discontinuation after 4 months.\nWithin 4 months, the patient complained of recurrence of grade 1 tumor pain and the MRI showed gradual tumor growth. At this time, radiotherapy was not considered appropriate given the young age and the proximity to the plexus nerves, instead medical treatment was discussed with the patient. Re-challenge with chemotherapy was not preferred by the patient, and an individual approach to medical treatment was sought. Based on the hyperperfusion of the patient’s tumor and the promising results of sorafenib in DF, we implied future treatment with bevacizumab 15 mg/kg q3wks., given the excellent tolerability profile of this agent.\nDuring the course of treatment, tumor pain disappeared and the patient experienced functional improvement. Initial radiological response was detected after 1 month, with a tumorshrinkage of 16 %. Treatment was continued for a total duration of 12 months with a further decrease in tumor diameter of 38 % (Fig. ). Further follow-up is performed by 3 months intervals by MRI and clinical visits. The first post-therapy scan confirmed tumor response in our patient, indicating sustained clinical activity. |
A 16-year-old girl presented with a history of sudden onset of pain in left leg with difficulty in walking for 2 days. The pain was intermittent and affecting her sleep. It was associated with intermittent abnormal upturned posture of the big toe. There was no history of swelling of leg. There was no history of any other abnormal movements. There was no history of similar complaints in the past. She had no history of any drug intake. On examination, her general physical examination was normal. Gait was antalgic. Higher mental function and cranial nerves were normal. Tone was increased below the knee on the left side. An upturned big toe without fanning of other toes was seen on the left []. This abnormal posture was sustained and increased on the sensory stimulus. Ankle jerk could not be elicited on the left side. A clinical diagnosis of a striatal toe was made and the patient was admitted for further evaluation [Figures and ]. The abnormal posture of the foot was maintained during sleep. Contrast-enhanced magnetic resonance imaging of the brain revealed no abnormality of the basal ganglia. Liver function test was normal and slit lamp examination did not show a KF ring. Corrected serum calcium was 9.8 mg/dL. There was no response to benzodiazepines. On the 3rd day after admission, she developed stiffness of bilateral lower limbs followed by episodic spasms of the back and neck. A clinical diagnosis of tetanus was made. There were no signs of autonomic hyperactivity and she did not have any seizures. On further probing, patient's mother revealed that she had suffered a fall a day before onset of her symptoms and hurt her nose. She had received tetanus toxoid (TT) 0.5 ml intramuscularly afterward. Her mother said that she had received her primary series of TT in infancy and received her last booster 6 years back at around 10 years of age. She was given tetanus immunoglobulin (TIG) 5000 units intramuscularly stat and also started on intravenous metronidazole and diazepam infusion. A full course of TT vaccination was also started. On the 5th day after admission, she developed mild trismus and opisthotonus completing the clinical picture of generalized tetanus. She did not develop any cranial nerve palsies or respiratory difficulty. She started showing signs of recovery by day 10 and made a complete recovery in 3 weeks from onset of symptoms. |
The presented case was a 31-year-old Chinese woman who was diagnosed with CED at the age of seven. Her antenatal and neonatal histories were uneventful. She had five siblings and she was the fourth child of her parents. Her eldest brother, who was 38 years old, developed CED symptoms at three years of age and the diagnosis was established six months following the initial presentation. Her parents and other siblings were asymptomatic; however, no investigation had been performed on them.\nAlthough her symptoms started at one year of age, due to financial constrained and poor social support, her parents brought her to seek medical treatment at the age of seven. At first, she presented with generalized muscle wasting of all four limbs, more prominently in lower extremities. Subsequently, she experienced progressive worsening of the bone pain. She had poor appetite and failure to thrive. At the age of seven, she was brought to the hospital due to unbearable lower limb pain. Physical examination revealed severe muscle and subcutaneous fat wasting. Her body weight was below fifth percentile. She walked with waddling gait. Femur radiograph was obtained and showed irregular sclerosis of the bone. Unfortunately, the image could not be obtained for review. Before the family history was disclosed and based on the clinical features and her femur radiograph, the initial diagnosis was muscular dystrophy with chronic osteomyelitis. Later, she was reviewed in pediatric clinic and her family history was obtained. Finally, the diagnosis of CED was made based on the clinical features, radiographic findings, and family history.\nAt the age of 20, she was also diagnosed with concomitant hypogonadism and primary hypothyroidism. Her thyroid gland had not been enlarged but her free T4 level was low and thyroid stimulating hormone level was high. The symptoms of hypothyroidism resolved with initiation of L-thyroxine. Her hearing was not compromised. During follow-up, serial chest radiographs were obtained and showed typical progressive diaphyseal hyperostosis of the humerus (, and ). The rib, clavicle, and vertebrae were spared. Her left radioulnar radiograph that was taken at the age of 24 years also showed similar changes (, and ). A computed tomography of orbit was performed recently due to progressive right exophthalmos. A lateral periorbital dermoid cyst was found but she was not eager for surgical removal. Moreover, the computed tomography showed classic skull changes of CED, which included extensive sclerosis and thickening of the frontal, temporal, and petrous bones, resulting in obliteration of diploe spaces and encroachment into the frontal and sphenoid sinuses. The maxillary sinus was relatively spared (). Her orthopantomogram (OPG) showed no hyperostosis of the mandible.\nSimilarly, her eldest brother had concomitant hypogonadism and primary hypothyroidism. His clinical presentation was similar to the patient but had manifested in more severe form. He was wheelchair-bound since the symptoms started due to severe muscle wasting and bone pain. He had bilateral mixed conductive and neurosensory hearing impairment. His physical examination showed mild hepatosplenomegaly.\nThe results of their full blood counts, serum urea, electrolytes, calcium, and liver function test were in normal range, except for persistently raised alkaline phosphates (180-265 U/L). They required long-term analgesia to relieve pain. Due to the disease progression and chronic bone pain, they were not able to adapt to normal social parameters. Both of them were unemployed and required social welfare support. |
A 28-year-old male was brought to the emergency department in a state of irritability following the history of ingestion of alcohol with snake venom, as narrated by his relative, one hour back. The patient was a snake charmer by occupation. The patient had a history of alcohol addiction for 15 years and had a history of repeated failed attempts to quit alcohol addiction in the past. On examination, pulse was 88 beats per minute, regular in rhythm and volume, having no special character, blood pressure was 120/70 mm of Hg and there were no signs of any bleeding tendencies. The patient was conscious and irritable, deep tendon reflexes were normal, bilateral plantar were flexor and there was no neuro deficit.\nThe abdomen was soft and non-tender, heart sounds were normal, the chest was bilaterally clear.\nThe patient’s whole blood clotting time was normal, random blood sugar was 80 mg/dl and the rest of the laboratory investigations including complete blood count, renal function test and liver function test were within normal limits. Upon providing injectable thiamine and 25% dextrose 100 ml, the patient improved clinically and became conscious and oriented. The patient then provided a history of snake venom addiction which began four months back, when he got divorced from his wife. It started as an addiction to snake bites which provided him the high sensation that he was using as an alternative to alcohol to reduce his alcohol consumption. Following a single snake bite, the patient used to get a high sensation for a period of 6-7 days, as a result of which his alcohol consumption reduced significantly. For the last one month, the patient was mixing his alcohol drinks with the venom of snakes in order to increase the duration of high effect with less amount alcohol. Cut-down, Annoyed, Guilt, Eye-opener (CAGE) score of the patient was calculated to be 3 out of 4 and the alcohol use disorder identifying test score was 26. The patient was started with cognitive behavioral therapy, chlordiazepoxide 10 mg hs (hora somni), along with a short serotonin reuptake inhibitor (escitalopram 10 mg once in a day). He improved clinically and was discharged after one week of admission in stable condition. He is currently doing well on follow-up with the de-addiction team of our hospital, participating actively in group therapies and motivational enhancement therapy. |
Mrs. X is a 62-year-old female with an ileostomy from prior hemicolectomy secondary to ischemic bowel who presented to our institution with an abrupt episode of painless bright red blood per ostomy. This patient has an extensive medical profile including a history of spontaneous portal vein thrombosis (PVT) that was complicated by splenic rupture and subsequent splenectomy as well as chronic portal hypertension, leading to cavernous transformation of stomal and abdominal wall varices. However, the patient was not treated with anticoagulation at the time of PVT diagnosis due to concurrent hemorrhagic stroke in the setting of suspected thrombocytopenic thrombotic purpura (TTP). She has had extensive investigations in the past regarding suspected autoimmunity, but no unifying diagnosis could be made.\nOn initial assessment, her hemoglobin (Hb) had dropped to 82 from a baseline of 110. The patient was transfused with 2 units of packed red blood cells (pRBCs) and her Hb improved subsequently to 104. Soon after, her bleeding ceased spontaneously, and she was admitted to the internal medicine service for further workup.\nOnce admitted, the patient underwent urgent endoscopic investigations which revealed scarring of esophageal varices that had been previously treated with sclerotherapy. The source of her hemorrhage was attributed to tortuous and dilated varices found on computed tomography (CT) imaging which extended throughout the ostomy defect and into the ostomy site (Fig. ). Shortly after, the patient experienced another episode of substantial hemorrhage through her ostomy and underwent emergent coil embolization to several branches of her stomal varices, achieving temporary hemostasis.\nThe patient experienced two recurrent episodes of major bleed in the days following embolization that required urgent transfusions before spontaneous hemostasis. Throughout her admission, the patient was assessed and closely followed by our general surgery team. However, after extensive discussions among experts, it was deemed that the peri-operative risks of surgical interventions were too great to warrant any elective procedure. This decision was made in context of a prior elective ileostomy reversal that was abandoned intra-operatively due to excessive bleeding. Our experts from vascular surgery, interventional radiology (IR), and gastroenterology also conducted independent assessments on the patient but unfortunately, no further surgical, radiological, or endoscopic interventions were deemed feasible for the correction of her stomal varices.\nGiven the lack of therapeutic options, we initiated the use of non-selective beta-blockade after reviewing case reports of its potential efficacy for minimizing recurrent stomal variceal hemorrhage in those with non-cirrhotic portal hypertension [, ]. Nadolol was started at a reduced dose due to patient's low baseline blood pressure and titrated to 40 mg oral daily as tolerated; reaching the targeted 25% decrease in heart rate. The patient was monitored closely over the next 2 weeks, while her diet was slowly advanced from nil per os (NPO) to diet as tolerated. She did not have any further hemorrhage through her ostomy and was discharged home with a safety plan in place. On follow-up, 3-month post-discharge, the patient reported no recurrent bleeds and her Hb was stable at her previous baseline. |
We present the case of a 77-year-old ambulatory man with hypertension, sarcoidosis, complete atrioventricular block status post-pacemaker implantation, chronic kidney disease due to FSGS, and right facial nerve paralysis, who presented with sporadic gait and right face numbness. He was diagnosed with sarcoidosis by biopsy of a tumor in front of the right tibia 14 years before presentation. Since the tumor and abdominal lymphadenopathy were the only manifestation of sarcoidosis and no other signs of organ involvement were present, he received no immunosuppressive treatment. The abdominal lymphadenopathy had been stable over time. Nine years before presentation, he was referred to our nephrology clinic to determine the cause of chronic kidney disease. His serum creatinine level was 1.2 mg/dL and he had proteinuria of 0.4 g per day. Hematuria was not present. Renal biopsy revealed six globally sclerotic glomeruli among all 34 glomeruli (18%) and some residual glomeruli with segmental sclerosing lesions, but no involvement of sarcoidosis. He was diagnosed with primary FSGS. Since the proteinuria was mild, he did not receive immunosuppressive treatment.\nOne year after that, the patient experienced palpitations and was diagnosed with complete atrioventricular block. Coronary angiography showed no significant stenosis of the coronary arteries, and he underwent pacemaker implantation. Whether sarcoidosis contributed to the complete atrioventricular block was unclear. The abdominal lymphadenopathy and the dyskinesia of the ventricular septum were stable and did not progress over time.\nThe patient was stable for eight years, until when he started to suffer from sporadic gait and right face numbness that occurred and resolved within a day every few weeks. Three months later, the symptoms recurred along with sudden dysarthria and left limbs weakness. Physical findings were notable for pronator drift on the left side. Perfusion computed tomography (CT) with iodinated contrast and CT angiography revealed no ischemic lesions or occlusion of major cerebral arteries. The symptoms disappeared three hours after the onset. A transient ischemic attack (TIA) was suspected, and he was admitted to the stroke unit. Ultrasonography revealed no stenosis of the internal carotid arteries, and transesophageal echocardiogram showed no abnormalities of the atrial septum. His pacemaker detected paroxysmal atrial fibrillation, which was presumed to be the etiology of the TIA. Thus, edoxaban 30 mg per day was started and he was discharged after one week of hospitalization.\nOne month after his discharge, his left leg started to swell and his gait worsened. Urinary protein excretion was 0.6 g per day, serum creatinine was at the baseline level of 1.6 mg/dL, and serum albumin level was 3.8 g/dL. Although no coagulopathy was found, ultrasonography revealed left femoral vein thrombosis that was 41 mm long. Edoxaban was stopped, and heparin was administered intravenously for two weeks. Low mobility due to his gait was presumed to be the cause of development of deep vein thrombosis (DVT). The patient was switched to warfarin and was discharged, but the left leg edema persisted. Three months later, he developed complications of urinary retention and constipation.\nFour months after discharge, the patient presented to the emergency department with sudden left leg pain and inability to walk. The entire left lower limb was slightly pale and had slow pitting edema. The left dorsal artery was not palpable, and the left femoral artery was barely palpable. Contrast CT revealed occlusion of the left femoral and superficial femoral arteries together with the known DVT in the left femoral vein (Fig. , ). Emergency thrombectomy for acute arterial occlusion was performed and the leg perfusion resumed. The emboli (maximum of 23 mm in diameter) were sent for pathological examination. The patient was admitted to the hospital and started on heparin infusion in place of oral warfarin. The history of recent TIA implied hypercoagulable state, but again no coagulopathy was found. While malignancy screening was being planned, the pathology of the arterial emboli revealed an unusual and surprising finding: the surface of the thrombi was filled with large atypical lymphoid cells (Fig. ) and was covering the necrotic interior of the thrombi. Immunohistochemical analysis showed that the tumor cells on the surface and the necrotic interior of the thrombi were positive for CD20 and CD79a but negative for CD3 (Fig. , ), which is characteristic of B cells. Leukocytosis was absent (white blood cell, 4,000/μL; segmented neutrophil, 55%; lymphocyte, 34%; monocyte, 9%; eosinophil, 2%). Serum soluble interleukin-2 receptor level was 1,548 U/mL (normal, 122–496 U/mL); lactate dehydrogenase (LDH) level, 808 U/L (normal, 120–245 U/L); LDH-2 fraction, 39% (normal, 28–35%), and LDH-3 fraction, 32% (normal, 21–27%). These findings were consistent with large B-cell lymphoma with intravascular proliferation, but the etiology of the aortic thrombi was unclear.\nThe hematology consultation team considered that the patient needed further biopsy to determine the etiology. Bone marrow biopsy showed normocellular marrow with normal maturation, but with infiltration of CD79a-positive large atypical lymphoid cells within the small vessels (Fig. , ). Although no lymphadenopathy was detected on palpation, CT scan showed swollen bilateral axillary and inguinal lymph nodes, which were up to 30 mm in diameter. While surgical biopsy of the right axillary lymph node and random skin biopsy were planned for diagnosis, the patient developed a complication of sepsis presumably due to pyelonephritis on hospital day nine. Piperacillin/tazobactam and vancomycin were started. Because partial thromboplastin time was prolonged, biopsies were withheld. Although white blood cell and neutrophil counts were improving, the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. The patient had a do-not-resuscitate order. His family agreed to an autopsy. |
A 71-year-old female presented with mild pharyngeal discomfort. Clinical examination revealed medial displacement of the left tonsil associated with transmitted pulsation of the surrounding pharyngeal wall. No cranial nerve palsy was evident. MRI showed a well-defined mass in the left parapharyngeal space. T\n1 weighted sequences demonstrated low signal intensity with multiple voids. On T\n2 weighted sequences the mass was of high signal intensity and revealed extensive enhancement following administration of gadolinium. Radiologic features were consistent with glomus vagale.\nIn light of her minimally disabling symptoms and the tumor being located on the side of her only seeing eye, the patient underwent a conservative “wait and rescan” plan of management. The tumor was observed with 8 serial MRI scans over a period of 7.4 years since 2006. This allowed a linear regression model to estimate the growth rates of its maximum axial dimension, maximum axial area, and volume.\nTumor size measurement was acquired on both hard copies and electronic images of MRI using a standardized method (). Dimensions were measured in 3 perpendicular axes. The largest dimensions in the anteroposterior (X) and mediolateral (Y) directions were measured on axial slices. If available, coronal or sagittal slices were utilized to measure the largest dimension in the craniocaudal direction (Z); otherwise it was estimated to be the product of slice thickness and number of slices in which the tumor could be identified. The maximum axial dimension was taken as X, which was the larger of the two dimensions on axial slice. Tumor was conceptualized to have an ellipsoid shape. The 3 perpendicular dimensions were used in the ellipsoid area and volume formulas to calculate the maximum axial area and volume of the tumor:\nIn order to standardize the measurement method on serial imaging, the baseline images were used for comparison to ensure that tumor dimensions were measured in the same orientation and on the same anatomical plane by identifying anatomical landmarks.\nOn serial MRI scans, the untreated glomus vagale demonstrated slow growth in a linear trend. No evidence of exponential growth was found. The maximum axial dimension revealed a growth rate of 0.68 mm/year (standard error 0.11 mm; p = 0.001; R\n2 = 87%), growing from 4.6 cm to 5.2 cm (13% growth) in a linear trend (). The maximum axial area illustrated a more stable trend with a growth rate of 0.04 cm2/year and a minimal growth from 10.8 cm2 to 11.0 cm2 (2% growth) (). The volume demonstrated a growth rate of 1.6 cm3/year growing from 32.5 cm3 to 47.1 cm3 (45% growth) in a linear trend (). Tumor doubling time was estimated to be 13.82 years using the following formula []:where T\n is tumor doubling time, T\n2 is last imaging time, T\n1 is first imaging time, V\n2 is volume at T\n2, and V\n1 is volume at T\n1. |
A 68-year-old male patient presented with a swelling and pain on the left pre-auricular region initiated about two to three years ago. He also had pain increases during opening his mouth. He had botulinum toxin injected on the affected area three month ago, and the symptom was not relieved at all. There was no particular underlined systemic disease in his medical record.\nA TMJ panoramic radiography, an imaging technique especially for assessing TMJ structure, was performed to evaluate if there is degenerative change of condyle and articulating eminence as well as relative position of condyle to articular fossa. The image revealed that joint space was slightly widened on left TMJ compared with the right side (). While, there was no restriction of both condyles during mouth opening (). No significant destruction or remodeling of bony structure was seen on joint components.\nA computed tomography (CT) and magnetic resonance (MR) imaging, both without contrast-media, were also obtained to evaluate any minor degenerative change of condyle and disc problem of TMJ. On the CT images, round soft tissue attenuation was localized on the posterolateral aspects of left condyle head (). The lesion showed soft-tissue equivalent attenuation compared with the adjacent muscles. As it was shown in the TMJ panoramic radiography, there was no significant bony destruction; however, slight thinning and focal discontinuity of cortical bone was detected on the condyle head contacting with the lesion. Also, mild subchondral sclerosis was found on this site ().\nOn MR image of proton density and T1-weighted sequence, the lesion was well-encapsulated and showed heterogeneous signal indicating solid mass rather than cyst (). The left mandibular condyle showed anterior dislocation due to a mass on the posterior aspect. However, TMJ discs were in normal position during the mouth open and close conditions. Local T1 hyper-intensity was recognized as it indicated that there was hematogenous component included in this lesion ().\nWith preliminary diagnosis of organized hematoma or giant cell tumor based on the images, the ovoid mass located on the postero-lateral aspect of left condylar head was surgically removed (). On the histopathologic exam, the lesion was composed of blood cells intermixed with granulation tissue. It was encapsulated with fibrous band and there was slight neovascularization was observed on the border of the capsule (). With the above findings, organized hematoma has been made as final diagnosis. No significant complications were found during a month of follow-up period. |
A 42-year-old man presented with a 13-year history of intractable tonic seizures that were indicative of right parietal lobe brain tumor. Plain computed tomography (CT) of the head revealed a tumor 40 mm in diameter and consisting of calcification and cyst formation located in the right parietal region. Magnetic resonance imaging (MRI) revealed an area of marked signal hypointensity around the solid component on T2 and T2 *-weighted imaging. These findings were thought to represent the calcification seen on CT, or perhaps hemosiderin deposition due to old hemorrhage. The tumor was located adjacent to the lateral ventricle, and the posterior part of the lateral ventricle was slightly enlarged toward the tumor. Normal choroid plexus of the right lateral ventricle was located in the normal position, and continuity of the normal choroid plexus to the tumor was not confirmed []. Cerebral angiography did not show any tumor staining or vascular abnormalities. The provisional diagnosis was cerebral cavernous angioma with hemorrhagic episode.\nA right parietal craniotomy was performed using a navigation system and motor-evoked potentials. A yellowish, granulomatous, moderately hard, slightly lobulated avascular tumor was located in the right parietal lobe, with scant hemosiderin deposition identified within the lesion. The margin of the tumor was covered with predominant gliosis. At the deepest part of the tumor, the tumor was firmly adhered to the subependymal layer of the lateral ventricle. During dissection of the adhered area, the ventricular ependyma was penetrated and the body of the lateral ventricle was visualized through the cavity of the removed tumor. The normal-appearing choroid plexus was placed within the posterior part of the lateral ventricle and continuity of the normal choroid plexus and the tumor was not confirmed. The lateral wall on the lateral ventricle showed a normal appearance and continuous coverage with ependymal []. The tumor thus originated completely in the cerebrum parenchyma and was firmly adhered to the wall of the lateral ventricle.\nPathological examination of the tumor revealed a papillary structure with a single layer of well-differentiated columnar epithelium in the lesion. Part of this lesion growth had infiltrated the cerebral parenchyma. In addition, activated macrophages were prominent around the cerebral parenchyma and were considered to represent a reactive lesion related to an old hemorrhage. Immunohistochemical examination was accomplished with the antibodies detailed in . Vimentin was strongly immunoreactive. Neural Cell Adhesion Molecule (N-CAM, CD56), Epithelial Membrane Antigen (EM) and Cytokeratin 7 (CK7) exhibited focal immunoreactivity. Cytokeratin 20 (CK20) and prealbumin were not immunoreactive. Glial Fibrillary Acidic Protein (GFAP) was strongly immunoreactive. Podoplanin exhibited focal immunoreactivity in a few reactive cells []. Mindbomb Homolog 1 (MIB1) labeling index (MIB1-LI) was 0.4%. Based on these findings, histological diagnosis was CPP with hemorrhagic episode.\nThe patient showed no postoperative neurological deficits, and cranial MRI confirmed complete removal of the tumor. Postoperatively, seizures were well controlled using antiepileptic drugs. |
A 58-year-old female patient with no known history of hyperlipidemia, hypertension, coronary artery disease, trauma or previous neck surgery was admitted to our clinic with a spontaneous pulsatile mass that caused swelling in the right jugular region. Computed tomography (CT) angiography was performed previously at another medical center and an 3.31 × 2.84 cm arterial aneurysm which was located distally to the bifurcation was revealed in right internal carotid artery (ICA) (). The neck of the ICA aneurysm was clearly demonstrated and cranial distance was enough to perform end to end anastomosis (4.64 cm) (). Treatment options were revised by our team and conventional surgery was selected for the patient. The first reason for this choice is the absence of additional surgical comorbidity. The second is that the head base distance of the aneurysm fits end to end anastomosis. The third is the risk of distal embolism, which may occur with the development of spontaneous aneurysm on the atherosclerotic ground, even if it is not observed in CT angiography results. Also there was no sign of pseudoaneurysm, the aneurysm size was sufficient and the patient’s will to get rid of the mass supported our decision.\nIn our case, it was thought that cranial perfusion would be sufficient because of good left carotid artery circulation. There is no consensus on the routine use of EEG and shunt applications previous studies. Therefore, EEG monitoring and shunt application were not used during the operation. Under general anesthesia the patient was prepped and draped, an incision was made through the medial border of the sternocleidomastoid muscle. Common carotid artery (CCA), ICA, external carotid artery (ECA) and aneurysm were exposed by protecting cranial nerves (). Carotid artery was clamped; the aneurysm was excised with blunt and sharp dissections. After removal of the aneurysm, proximal and distal ends of the ICA were seen to be in sufficient length. Proximal and distal ends of the ICA were repaired by end-to-end anastomosis without vein graft. A specimen of aneurysm wall was sent to pathology department and the results were well-matched with a true aneurysm. An antiplatelet treatment consisting of 1 drug was initiated (acetylsalicylic acid 100mg) and was continued after the discharge. There were no complications in postoperative period and she was discharged uneventfully on the fifth postoperative day. A CT angiography examination was carried out 12 weeks after the surgery (). Flow was sufficient in control angiography and no stenosis or re-developing aneurysm was observed in the right carotid artery.\nThere is no consensus on the routine use of EEG and shunt applications in the studies conducted. In our case, it was thought that cranial perfusion would be sufficient because of good left carotid artery circulation. Therefore, EEG monitoring and shunt application were not used during the operation |
A 62-year-old man presented at the Neurology consultation with a six-week history of a severe, strictly left orbitotemporal headache, with a frequency of three attacks per week, occasionally more than one at the same day. Most of them occurred in the first half of the night, waking him up, and lasted between thirty minutes and one hour. He used to take ibuprofen as acute treatment, with unsatisfactory response, since he did not notice a significant difference between treated and untreated attacks in terms of duration and pain intensity. To relief the pain, he used to open the window to get some fresh air. The headache was always associated with ipsilateral conjunctival injection and lacrimation. Pain triggers were not identified by the patient. He had no personal or familial history of headaches. His medical history was remarkable for hypertension and asthma, with a past surgical history including septoplasty and bilateral middle turbinectomy and uncinectomy due to nasal respiratory insufficiency. By the time of medical evaluation he was asymptomatic and neurological exploration was unremarkable. The clinical picture was suggestive of a CH and the patient was medicated with verapamil 120 mg daily. A MRI scan was performed, which revealed a sphenoid sinus mucocele, without secure expansion of the sinus. Two weeks later the patient came to the Emergency Department with complaints of horizontal diplopia that he noted when he woke up in that morning. He maintained the headache attacks, with similar characteristics, despite prophylactic therapy. Neurological examination revealed left eye adduction palsy and ptosis. A brain CT scan was performed and excluded lesions other than the mucocele. Paranasal sinus MRI revealed molding of the medial wall of left cavernous sinus by the sphenoid mass (). A paranasal sinus CT scan was also performed to allow for a better characterization of the lesion, showing sclerosis and interruption of the roof and posterior wall of the left sphenoid hemisinus (). The patient was submitted to surgical drainage of the mucocele by transnasal-transphenoidal approach, with complete resolution of the adduction impairment, persisting a mild left eye ptosis. After the surgery the attacks stopped, and in the six-month follow-up he reported no further attacks. |
A 60-year-old male was referred to our department, complaining about gradually worsening hoarseness, during the last 8 month period. Occasional dysphagia and foreign-body sensation were also reported upon referral. The patient was a heavy smoker for more than 20 years, reporting an average of 20 cigarettes per day. Alcohol was also a factor, and although no real alcohol abuse or indulgence was noted, the patient was a rather frequent user.\nMedical history only revealed arterial hypertension under treatment with beta blockers. Haematological and biochemical tests did not show any significant abnormalities.\nPhysical examination included a full head and neck examination, complemented with flexible fiberoptic laryngoscopy. Typical ear, nose and throat examination did not reveal any abnormal findings and neck palpation was negative. However, fiberoptic laryngoscopy revealed a lesion affecting both vocal cords and anterior commissure, while vocal cord mobility appeared impaired. On these grounds, a cervicothoracic and upper abdomen computed tomography (CT) scan with intravenous gadolinium was decided and the patient was scheduled for direct microlaryngoscopy and biopsy of the lesion under general anaesthesia.\nImaging confirmed the laryngeal lesion, yet it also indicated a second lesion about 2 cm below the inferior end of the primary one, arising somewhere between the first and second tracheal ring. Intermediate tissue appeared grossly normal (). No signs of enlarged cervical lymph nodes were noted and laryngeal cartilages showed no abnormal findings.\nOn the other hand, histopathological examination after biopsy of the lesion under general anaesthesia confirmed the diagnosis of squamous cell carcinoma. The lesion was carefully mapped and proved to be a glottic carcinoma affecting the anterior commissure and appearing in strong correlation with the thyroid cartilage. The lesion infiltrated the left and the first tertile of the right vocal cord. No subglottic extension was noted. In this context, the patient was informed and consent for radical surgical therapy was obtained.\nThe patient underwent total laryngectomy and wide excision of the trachea which included the second tumour within safe limits (). The procedure was complimented with left thyroid lobectomy and bilateral selective neck dissection (Robin’s levels II–IV). Paratracheal lymph nodes (Robin’s level VI) were also carefully dissected. The overall postoperative course was uneventful. The patient was discharged from our department on day 16 with very good swallow function and was decannulated after 1 week. Surgical resection was followed by postoperative radiation therapy (6400 cGy/32 fraction).\nThe final pathological report was of crucial importance in our case. First of all, the surgical margins of resection were found to be free of disease. Second, histological sections from the tumour of the glottis showed the characteristic morphology of squamous cell carcinoma. Cancer cells were large in size and polygonal in shape with eosinophilic cytoplasm and nuclei with moderate variation in size and shape. There were a moderate number of mitoses and keratinisation could be focally observed. Cancer cells showed an infiltrative pattern consisting mainly of nests and trabeculae that invaded the vocalis muscle in both the vocal cords. The perichondrium of thyroid cartilage was focally invaded by cancer cells. Histological sections from the tumour of the trachea showed morphological features identical to those of the tumour of the glottis. An upward infiltrating pattern could be noticed. Moreover, a comparative immunohistochemical study of the two tumours showed strong positivity of cancer cells in stains for keratins AE1/AE3 and 34βΕ12 and moderate positivity in stains for CK5/6, CK8/18 and epithelial membrane antigen. Immunohistochemistry for D2-40 antigen (podoplanin) illustrated the positivity of the lymphatic endothelium. Immunohistochemical stains for other vascular endothelia (CD31 and CD34 antigens) were also performed, and were negative. In the region between the two tumours, many lymphatics containing neoplastic emboli could be observed (). Finally, two tumour-infiltrated lymph nodes (the larger being of 1.2 cm diameter) with extracapsular spread were found in the left neck dissection specimen. A pT4a(m)N2b stage, according to eighth edition TNM staging, was established. |
Our patient was a 24-year-old Albanian man who was admitted to the Emergency Department of the University Hospital in Kosovo in severe hemorrhagic shock due to a massive hematuria. The patient is a farmer, does not smoke, and does not consume alcohol. He has no significant family and social history of medical relevance. Ten years ago, he had sustained a third-degree burn injury over approximately 70% of his body surface area and had been treated in a specialized center in a neighboring country. During that hospitalization, the patient had multiple venous lines placed in the groin and developed an infection that led to the rupture of the common femoral artery. To treat it, an autogenous vein extra-anatomic iliofemoral bypass was constructed. Since then, he had never been seen by a vascular surgeon.\nPrior to his admission to our emergency department, he had two episodes of gross hematuria for which he was treated at the regional hospital. Diagnostic evaluation during earlier hospitalizations did not reveal the cause of bleeding. AUF was not considered on either of the occasions. The treatment was conservative and involved bladder lavage and blood transfusions. He was discharged on antibiotics, uroseptics, and iron supplements.\nThe possibility of communication between the arterial and urinary tracts was suspected on the basis of cystoscopy performed in the outpatient setting 2 days before the current admission (Fig. ). The finding of the source of the bleeding at the right upper corner of the bladder, very close to the ureteral opening, raised the suspicion of possible AUF. The diagnosis was confirmed the next day, when contrast-enhanced magnetic resonance imaging showed proximity of a small pseudoaneurysm located at the proximal anastomosis of the enlarged extra-anatomic right iliofemoral autogenous vein graft and bladder (Fig. ). The patient, who was free of bleeding, was referred to a vascular surgeon and admitted to the vascular surgery department. Several units of red blood cells and fresh frozen plasma (FFP) were ordered, along with antibiotics, and the patient was scheduled for elective surgery on the next day. Unfortunately, during the night, he experienced a third episode of exsanguinating bleeding and was transferred to the emergency department.\nAt the emergency department, he was confused and anxious, and his skin was pale, cold, and clammy. He was sweating and was breathing rapidly. His pulse on the peripheral arteries was weak, rapid, and thready. His fingernails and lips were blue, and his capillary refill time was 5 seconds. His blood pressure was 70/40 mmHg, heart rate 130 beats/minute, and peripheral capillary oxygen saturation 92%. His hematocrit was 19%, and his hemoglobin was 7 g/dl. His platelet count was normal, and his white blood cell count was slightly elevated (11.7 × 109/L). His glucose, cholesterol, urea, and creatinine concentrations were within normal range. His total bilirubin was moderately elevated (30.6 μmol/L), and his transaminase level was normal. He had a significantly high level of C-reactive protein (55.8 mg/L). His urine was full of blood cells. No serology or microbiology was performed. Hemodynamic resuscitation was initiated immediately. Two large-bore (16-gauge) intravenous catheters were inserted. Crystalloids and colloids were rapidly administered, and red blood cells and FFP were ordered.\nInduction agents etomidate (0.3 mg/kg), fentanyl (3 μg/kg), and rocuronium (1.2 mg/kg) were administered. The patient was intubated and escorted to the operation room. Anesthesia was maintained with sevoflurane (0.7–1.3 minimum alveolar concentration), atracurium, and fentanyl. To achieve hemodynamic stability, vasopressors (dopamine 5–7 μg/minute) were used until several units of red blood cells and FFP were brought from the transfusion desk. To minimize the possibility of rebleeding, permissive hypotensive resuscitation was maintained.\nThe abdomen was opened employing a right extraperitoneal approach. After obtaining vascular control, the rupture site was reached through the native aneurysmal part of the venous graft and was closed with simple sutures (Fig. ). Because of the severe scars on the skin and varicosity of the saphenous vein, we decided to perform a new bypass using a synthetic graft. The proximal anastomosis of the synthetic graft was placed on the iliac artery 5 cm above the site of the rupture, and the distal part of the graft was anastomosed in an end-to-side fashion with the existing autogenous vein graft, several centimeters before the site of the original distal anastomosis. The graft above the distal anastomosis was ligated (Fig. ).\nAt the end of the operation, the patient was transferred to the intensive care unit. On the next day, he was extubated and transferred to the ward. The patient recovered completely, and postoperative cystoscopy showed no signs of pathological communication (Fig. ). He was free of hematuria episodes for the whole postoperative period. Computed angiography performed 2 years after the surgery showed correct position of the graft with no complications (Fig. ). |
We present the case of a 77-year-old ambulatory man with hypertension, sarcoidosis, complete atrioventricular block status post-pacemaker implantation, chronic kidney disease due to FSGS, and right facial nerve paralysis, who presented with sporadic gait and right face numbness. He was diagnosed with sarcoidosis by biopsy of a tumor in front of the right tibia 14 years before presentation. Since the tumor and abdominal lymphadenopathy were the only manifestation of sarcoidosis and no other signs of organ involvement were present, he received no immunosuppressive treatment. The abdominal lymphadenopathy had been stable over time. Nine years before presentation, he was referred to our nephrology clinic to determine the cause of chronic kidney disease. His serum creatinine level was 1.2 mg/dL and he had proteinuria of 0.4 g per day. Hematuria was not present. Renal biopsy revealed six globally sclerotic glomeruli among all 34 glomeruli (18%) and some residual glomeruli with segmental sclerosing lesions, but no involvement of sarcoidosis. He was diagnosed with primary FSGS. Since the proteinuria was mild, he did not receive immunosuppressive treatment.\nOne year after that, the patient experienced palpitations and was diagnosed with complete atrioventricular block. Coronary angiography showed no significant stenosis of the coronary arteries, and he underwent pacemaker implantation. Whether sarcoidosis contributed to the complete atrioventricular block was unclear. The abdominal lymphadenopathy and the dyskinesia of the ventricular septum were stable and did not progress over time.\nThe patient was stable for eight years, until when he started to suffer from sporadic gait and right face numbness that occurred and resolved within a day every few weeks. Three months later, the symptoms recurred along with sudden dysarthria and left limbs weakness. Physical findings were notable for pronator drift on the left side. Perfusion computed tomography (CT) with iodinated contrast and CT angiography revealed no ischemic lesions or occlusion of major cerebral arteries. The symptoms disappeared three hours after the onset. A transient ischemic attack (TIA) was suspected, and he was admitted to the stroke unit. Ultrasonography revealed no stenosis of the internal carotid arteries, and transesophageal echocardiogram showed no abnormalities of the atrial septum. His pacemaker detected paroxysmal atrial fibrillation, which was presumed to be the etiology of the TIA. Thus, edoxaban 30 mg per day was started and he was discharged after one week of hospitalization.\nOne month after his discharge, his left leg started to swell and his gait worsened. Urinary protein excretion was 0.6 g per day, serum creatinine was at the baseline level of 1.6 mg/dL, and serum albumin level was 3.8 g/dL. Although no coagulopathy was found, ultrasonography revealed left femoral vein thrombosis that was 41 mm long. Edoxaban was stopped, and heparin was administered intravenously for two weeks. Low mobility due to his gait was presumed to be the cause of development of deep vein thrombosis (DVT). The patient was switched to warfarin and was discharged, but the left leg edema persisted. Three months later, he developed complications of urinary retention and constipation.\nFour months after discharge, the patient presented to the emergency department with sudden left leg pain and inability to walk. The entire left lower limb was slightly pale and had slow pitting edema. The left dorsal artery was not palpable, and the left femoral artery was barely palpable. Contrast CT revealed occlusion of the left femoral and superficial femoral arteries together with the known DVT in the left femoral vein (Fig. , ). Emergency thrombectomy for acute arterial occlusion was performed and the leg perfusion resumed. The emboli (maximum of 23 mm in diameter) were sent for pathological examination. The patient was admitted to the hospital and started on heparin infusion in place of oral warfarin. The history of recent TIA implied hypercoagulable state, but again no coagulopathy was found. While malignancy screening was being planned, the pathology of the arterial emboli revealed an unusual and surprising finding: the surface of the thrombi was filled with large atypical lymphoid cells (Fig. ) and was covering the necrotic interior of the thrombi. Immunohistochemical analysis showed that the tumor cells on the surface and the necrotic interior of the thrombi were positive for CD20 and CD79a but negative for CD3 (Fig. , ), which is characteristic of B cells. Leukocytosis was absent (white blood cell, 4,000/μL; segmented neutrophil, 55%; lymphocyte, 34%; monocyte, 9%; eosinophil, 2%). Serum soluble interleukin-2 receptor level was 1,548 U/mL (normal, 122–496 U/mL); lactate dehydrogenase (LDH) level, 808 U/L (normal, 120–245 U/L); LDH-2 fraction, 39% (normal, 28–35%), and LDH-3 fraction, 32% (normal, 21–27%). These findings were consistent with large B-cell lymphoma with intravascular proliferation, but the etiology of the aortic thrombi was unclear.\nThe hematology consultation team considered that the patient needed further biopsy to determine the etiology. Bone marrow biopsy showed normocellular marrow with normal maturation, but with infiltration of CD79a-positive large atypical lymphoid cells within the small vessels (Fig. , ). Although no lymphadenopathy was detected on palpation, CT scan showed swollen bilateral axillary and inguinal lymph nodes, which were up to 30 mm in diameter. While surgical biopsy of the right axillary lymph node and random skin biopsy were planned for diagnosis, the patient developed a complication of sepsis presumably due to pyelonephritis on hospital day nine. Piperacillin/tazobactam and vancomycin were started. Because partial thromboplastin time was prolonged, biopsies were withheld. Although white blood cell and neutrophil counts were improving, the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. The patient had a do-not-resuscitate order. His family agreed to an autopsy. |
A 63-year-old female was referred to the pulmonary clinic for insidious onset of dyspnea. The patient had multiple medical problems, including inflammatory bowel disease treated with prednisone varying from 10 to 60 mg/day, hypertension, hypothyroidism, peptic ulcer disease, and a distant history of lower extremity DVT. She was also using continuous positive airway pressure at night for obstructive sleep apnea with good control of her symptoms.\nShe had gradual onset of her dyspnea around one year prior and it had progressed to the point where she was unable to complete activities of daily living without getting short of breath. She was not hypoxic at rest with a room air oxygen saturation of 95%. Her physical examination was unremarkable. The only abnormality in her spirometry was an expiratory reserve volume of 4% predicted. The diffusion capacity was in the normal range. Arterial blood gas analysis showed a PaO2 of 66, PaCO2 of 38, and pH of 7.41. CT scanning of the chest including HRCT, inspiratory and expiratory films, and CT angiogram did not show parenchymal lung disease. Echocardiogram showed normal left atrium, right atrium, and right ventricle with a left ventricular ejection fraction of 43%. There was mild diffuse hypokinesis and a dobutamine stress echocardiogram did not show regional wall motion abnormality or valvular disease. As her symptoms were out of proportion to pulmonary abnormalities, left and right heart catheterization were done. Left heart catheterization confirmed normal coronaries and mild LV dysfunction. PA pressures were 21/7 mm Hg and PAOP was normal. Cardiac output obtained by thermodilution method was 5 liters/min. PA saturation was 69.4%.\nAs her symptoms worsened an echocardiogram with bubble study was done that showed early appearance of bubbles in the left atrium suggesting intracardiac shunting. Right to left shunt was confirmed on TEE. On augmentation of her heart rate by dobutamine and atropine, a significant increase in the extent of right to left shunting was seen on contrast injection. At transcatheter closure of the PFO, the size of the defect was found to be 7 mm by sizing balloon. The defect was closed with a 12 mm Amplatzer septal occluder device with no residual shunting by color Doppler at the end of the procedure. After the closure she had noticeable improvement in the exercise tolerance and O2 saturation. Serial echocardiograms did not reveal recurrence of the shunt or displacement of the closure device. |
A 52-year-old female with end-stage renal disease on hemodialysis, hypertension, hyperlipidemia, coronary artery disease, heart failure with preserved ejection fraction, and insulin-dependent type 2 diabetes mellitus presented with 2 weeks of right upper extremity swelling. The swelling was attributed to right subclavian vein stenosis, presence of right internal jugular tunneled dialysis catheter, and functional right brachial-antecubital arteriovenous fistula. She was scheduled for removal of the right dialysis catheter, placement of left dialysis catheter, central venography with possible angioplasty and stenting of the right subclavian vein under general anesthesia.\nAfter uncomplicated induction of anesthesia, the patient was anticoagulated with 4000 units of intravenous heparin. The right internal jugular dialysis catheter was removed over a wire placed into the superior vena cava. A large clot was detected in the right subclavian vein, which was treated with angioplasty via the cephalic vein using a 14-mm balloon, resulting in improvement in venous flow. Both access points were closed, and anticoagulation was reversed with 25 mg protamine sulfate 65 minutes after initial heparin bolus. The surgical team then encountered resistance while placing a microwire via the left internal jugular vein into the left subclavian vein for left dialysis catheter placement. After several attempts, the wire was placed under fluoroscopic guidance and the dilator was advanced. During dilator advancement, seen in , resistance was encountered at the cavoatrial junction. Immediately thereafter, the patient experienced profound hypotension and received cardiopulmonary resuscitation (CPR). Intraoperative transesophageal echocardiography imaging demonstrated pericardial tamponade with partial clot. The surgeon obtained large bore vascular access and placed an arterial line in the femoral artery. CPR was stopped after return of spontaneous circulation, with the patient being responsive to vasopressors and further resuscitated with 9 units packed red blood cells, 9 units fresh frozen plasma and 2 units of platelets.\nThe trauma service attending was paged emergently to relieve tamponade and evacuate the clot. A sternotomy was performed left of midline that transected four ribs inferior to the sternal notch. The pericardium was opened and blood with clot was removed with improvement in hemodynamics. Upon arrival of the cardiothoracic team, the mediastinum was explored and a minimally bleeding lesion in the left subclavian vein was identified and closed. On further examination, there was a nonbleeding puncture in the left pulmonary trunk in proximity to the breach in the subclavian. This was the presumed etiology of tamponade and was reinforced with a single pledgeted suture.\nA left internal jugular tunneled dialysis catheter was placed under direct visualization with the tip below the cavoatrial junction for dialysis in the postoperative period. Mediastinal drains were placed, and the sternum was closed with two titanium plates, screws and wires. Chest x-ray can bee seen in .\nPatient was discharged on post-op day 7, however had a complicated course post discharge with readmissions for central venous thrombosis, pulmonary emboli, sternal dehiscence with osteomyelitis secondary to coagulase negative staphylococcus, bacteremia with the same organism, complicated hardware extraction with sternal reconstruction and flaps. Overview of complications can be seen in . At time of writing, the patient had been seen at outpatient follow-up and was doing well, with removal of her tunneled dialysis catheter and a working right upper arm arteriovenous fistula.\nInformed consent for publication was obtained from patient per institutional policies, and sufficiently deidentified to protect privacy. |
Our patient was a 74-year-old man of Middle Eastern ethnicity with a previous medical history including hypertension, benign prostatic hyperplasia, polycythemia vera, and duodenal ulcers. He received metoprolol (100 mg daily) as well as aspirin (75 mg daily), had a history of tobacco smoking, but ceased tobacco use 15 years prior to the current admission. He did not consume alcohol. He was born in Iraq and arrived in Sweden 2002. He was previously employed as a medical secretary but he had retired at the time of admission. His previous social history is largely unknown, but he was married, and he had three children (two sons and one daughter) according to previous medical files.\nIn the early spring of 2019, he developed dyspnea and flank pain, and following investigations at a secondary care institution, a subsequent radiological computed tomography (CT) examination revealed at least six lesions in his liver, of which the largest deposit measured 13 mm. In addition, a 50 mm large soft tissue expansion in conjunction to his chest wall with engagement and destruction of the eighth rib was observed. In his lungs, 13 nodular lesions were detected of which the largest measured 8 mm. A bone metastasis to the right acetabular region was also visualized, in addition to a 10 mm large lesion in his left adrenal gland. The latter nodule was present already on a radiological examination performed 12 years earlier and found stationary with no increase in overall size; it was therefore believed to be benign. To summarize, the lesions observed were highly suspicious for metastatic deposits from a CUP. An ultrasonography-guided core-needle biopsy of the chest wall lesion was performed, and a diagnosis of metastatic NEC was rendered. The material was immediately sent for second opinion consultation at our tertiary unit.\nThe histopathological examination revealed a tumor with a predominant solid growth pattern, diffusely infiltrating the surrounding fibrotic stroma (Fig. a). The tumor cells displayed round to slightly polygonal nuclei, focally pleomorphic, with an evenly distributed chromatin (Fig. b). Several nuclear inclusions were observed, as were comedo-like tumor necrosis and apoptotic bodies. No prominent nucleoli or cytoplasmic pigmentation was noted. The immunohistochemical analysis was carried out in a routine pathology laboratory setting with clinically accredited antibodies and standardized methodology. The profiling was consistent with a tumor uniformly positive for SYP and CD56 (Table ; Fig. c, d). Unexpected and diffuse cytoplasmic immunoreactivity was noted for insulinoma-associated protein 1 (INSM1), and subsets of cells were also positive for ISL LIM homeobox 1 (ISLET1; Fig. e, f). The tumor was negative for melanoma antigen (Melan A) and human melanoma black 45 (HMB45; Fig. g, h) but was strongly positive for vimentin (Fig. i). Scattered tumor cells were positive for cytokeratin OSCAR. The tumor cells were negative for: pan-cytokeratin (CK MNF 116); cytokeratins 5, 7, 19, and 20; epithelial membrane antigen (EMA); GATA binding protein 3 (GATA3); hepatocyte-specific antigen; P63; prostate-specific antigen (PSA); thyroid transcription factor 1 (TTF1); paired box 8 (PAX8); caudal type homeobox 2 (CDX2); carcinoembryonic antigen (CEA); CgA; secretagogin; steroidogenic factor 1 (SF1); inhibin alpha; glucagon-like peptide 1 (GLP1); and pancreatic and duodenal homeobox 1 (PDX1) (data not shown). The Ki-67 index was 50%. The P53 immunoreactivity was focal and weak, not suspicious for an underlying TP53 gene alteration. The pathology report concluded the presence of a metastatic, high-grade malignant tumor with a neuroendocrine profile and unknown primary location; the absent CgA immunoreactivity could argue in favor of a NEC because this tumor entity occasionally downregulates CgA expression upon tumoral de-differentiation. The keratin-negative profile did, however, also raise the suspicion of a non-NET with a neuroendocrine differentiation, but the paraffin block was exhausted after cutting material for immunohistochemistry; therefore, no additional analyses could be performed.\nOur patient was discussed at a multidisciplinary conference and was recommended to commence chemotherapy (carboplatin and etoposide). On admission to our hospital, he was confined to a wheelchair and in considerable pain from his groin and lumbar regions. His groin was investigated, and a 20 mm enlarged lymph node was palpable and was assumed to be part of his disseminated disease. He had substantial bilateral pitting edema over his lower extremities but was without dyspnea. He had no fever or symptoms indicating an infection. No neurological examination was performed. His blood pressure was measured several times during the hospitalization, but was 123/89 mm Hg shortly after admission, with a pulse rate of 113 and a saturation of 93% without oxygen supply. His hemoglobin count was 170 gram/L (reference, 134–170), his erythrocyte count was 5.5 (× 1012/L) (reference, 4.2-5.7), his leukocyte count was 8.0 (× 109/L) (reference, 3.5-8.8), and his thrombocyte count was 117 (× 109/L) (reference, 145–348). Liver parameters were mostly normal (aspartate aminotransferase, alanine aminotransferase, gamma-glutamyl transferase, and bilirubin), except for hypoalbuminemia (24 g/L, reference, 34–45). His renal function was not impaired, as made evident by a normal plasma creatinine and a calculated glomerular filtration rate (GFR) of 75 mL/minute (reference, > 60 mL/minute). Following the first round of chemotherapy, he developed partly therapy-resistant back pain, and was planned for external radiation directed at his pelvis and lower back for palliative purposes. Besides the chemotherapy treatment, he was administered fentanyl (75 micrograms/hour, transdermal administration) for his back pain, ondansetron (4–8 mg intravenously) against chemotherapy-induced nausea, and sodium phosphate laxatives against morphine-instigated constipation. He was also administered furosemide intravenously (20–40 mg) when needed. Cortisone (betamethasone) was also administered orally using a standardized gradual reduction scheme.\nShortly afterwards, he developed a left-sided, pathological hip fracture (Fig. a). Following surgery, the excised femoral head and neck was sent for histopathological examination. Histological evaluation of material decalcified by formic acid and microwave treatment revealed infiltration by a nest-forming tumor with large atypical nuclei displaying multiple nucleoli and a partly loose chromatin (Fig. b, c). Nuclear inclusions were noted. The mitotic rate was 20 mitoses/10 high-power fields. Tumor necrosis was noted (Fig. b). Immunohistochemistry revealed partial expression of SYP, Melan A, and HMB45 (Table ; Fig. d–f), as well as positive immunoreactivity towards SOX10 (Fig. g). Moreover, vimentin immunoreactivity was noted, as well as absent staining of CK MNF 116, CK7, CK20, desmin, myogenin, CD10, and CgA (data not shown). The diagnosis was consistent with a metastatic epithelioid malignant melanoma with neuroendocrine differentiation.\nHe subsequently developed bilateral pleural effusions, and despite intermittent treatment with pleurocentesis, he developed respiratory failure, which was aggravated by the occurrence of lobar pneumonia. Given his status, no clinical investigations regarding the occurrence of an undiagnosed primary cutaneous or mucosal malignant melanoma were initiated. Ultimately, he developed septicemia and died only 3 months after initial presentation. No autopsy was performed. |
A 2-months-old Brazilian girl presented to the physiotherapy department diagnosed with congenital hydrocephalus. The hydrocephalus was detected before delivery, and she was born at 37 gestational weeks by cesarean section. No similar familiar medical history or genetic information was mentioned at the assessment.\nOn her first day of life, she underwent ultrasonography (US) and magnetic resonance imaging (MRI) examinations to determine the cause of the hydrocephalus. According to the US, she presented with an increase in the volume of the lateral and the third ventricles and thinning of the corpus callosum. The MRI showed a round lesion measuring 0.5 cm in the cerebral aqueduct isointense to the encephalic parenchyma without contrast enhancement, significant increase of the posterior portion of lateral ventricles, thinning of cerebral cortex, and increase of the third ventricle. At 14 days of age, she underwent implantation of a ventricular peritoneal shunt. At 2 months of age, she was assessed by a physiotherapist to initiate an intervention to prevent developmental motor delays. The assessment addressed anamnesis and a physical evaluation using the CME motor scale and the Alberta Infant Motor Scale (AIMS).\nThe CME motor scale is composed of 41 items to assess motor development through automatic motor reactions. The response to each item is graded between 0, indicating no response, and 3, indicating complete reaction. Results provide a child's developmental motor age and can be used to create a personalized treatment plan.\nThe AIMS is a gross motor observational tool that evaluates the control of antigravitational muscles in various postures. The AIMS is a validated scale used in clinical practice and research. It can detect developmental delays or abnormalities, being able to identify mild changes in the motor development and to measure intervention effectiveness.\nThe assessments were conducted by an experienced physiotherapist unrelated to the intervention to avoid any influence in the outcomes. They were repeated at 3, 6, 9, and 16 months of chronological age to determine acquisition of developmental milestones and to observe treatment results. In order to interpret the outcomes properly, chronological age was corrected for prematurity. |
A 65-year-old female presented with a chief concern of left abdominal wall pain and possible incisional hernia. She had undergone a left radical nephrectomy, splenectomy and distal pancreatectomy earlier in the year for a mass on the left kidney. Pathologic evaluation revealed the mass to be a large perinephric abscess of uncertain etiology. Her recovery was complicated by a wound infection and development of a pancreatic fistula, which was repaired. A few months later, after recovery, the patient began experiencing significant pain at the incision site, which was worse with daily activity. She denied any symptoms of infection, such as fever or chills. A complete blood count performed shortly after surgery revealed no leuckocytosis. She was referred to general surgery for a suspected incisional hernia. Physical examination revealed a firm bulge at the superior aspect of the incision in the left upper quadrant, which was tender to palpation and non-reducible. There was no erythema, fluctuance or other signs of infection. An abdominal computed tomography (CT) scan revealed muscular laxity and a questionable abdominal wall hernia at the incision site (Fig. ). The patient was taken to the operating room for abdominal wall wound exploration and possible hernia repair. The superior aspect of the previous incisional scar was reopened and dissection carried down through the subcutaneous tissue to the level of the fascia. No fascial defect was found; however, a firm mass was palpated within the abdominal wall. Further dissection revealed a malpositioned left rib surrounded by fibrous tissue. The rib appeared devitalized, thin and fragile. We then referred back to the CT scan and in this location, no calcification could be observed. However, this was clearly a rib. The rib was dissected until a healthy portion was noted and the devitalized rib was resected (Fig. ). The wound was surveyed again for any muscular or fascial defects, none were found. The patient was discharged in good condition the same day. Removal of the rib sequestrum resolved the patient's symptoms. |
A 3-year-old male patient of Cambodian origin was referred for extraction of an unusual right maxillary incisor after a traumatic injury. According to his mother, there were no such anomalies in the other family members. The anamnesis revealed that the child was born premature and actually presented with delayed growth, hyperlaxity, and cerebellar atrophy. Moreover, his built was small for his age. These symptoms led to genetic exploration of syndromic diseases, but with no remarkable findings.\nThe extraoral examination did not show any alterations. Intraoral examination revealed a gingival laceration next to a decayed triple tooth, in which was a double crown in place of the maxillary central incisor fused with the lateral incisor (Figure ). These triple teeth were affected by a large carious lesion at the junction between the double crown and an incipient carious lesion in the groove between the double crown and the lateral incisor crown. These teeth presented no mobility or fracture. In the region of the right central incisor, a submucosal abscess was observed, suggesting infected pulp necrosis. Moreover, the left mandibular central and lateral incisors appeared fused, with a unique but larger crown (Figure ). No other findings were reported.\nAn intraoral periapical radiograph of the triple teeth revealed two distinct structures, an upper right incisor with a possibly unique pulp chamber (difficult to confirm because of the carious lesion) and a large root canal (possibly dividing into two canals in the middle-third), and a lateral incisor with separate pulp chamber and separate root canal (Figure ). It also showed a radiolucent area around the apex of the large central incisor, but the lateral incisor seemed unaffected. It was not possible to take a radiograph of the double teeth due to noncompliance of the patient.\nThe diagnosis was gingival laceration due to trauma and abscess on the triple teeth due to an advanced carious lesion. Because of the difficulty in performing root canal treatment in such teeth and the poor cooperation of the young patient, extraction of the triple teeth was planned. Due to the difficulty in extracting such teeth and for the patient's comfort, this procedure was performed under nitrous oxide/oxygen inhalation. To prevent functional, esthetic, and phonetic problems, the missing teeth should have been replaced with a transitional partial denture. However, the replacement was not possible at this stage due to insufficient cooperation by the patient. A 1-year follow-up showed good healing (Figure ). It was however not possible to perform a radiograph to check the underlying tooth germs, because of insufficient cooperation.\nThe extracted teeth had three separate crowns and roots conjoined from the crown to the apex and possibly from the incisal edge to the apex in the large central incisor (Figure ). Macroscopically, almost no root resorption was detected. The teeth were analyzed using micro-computed tomography to obtain a three-dimensional model and a two-dimensional cross-sectional slice (Figure ). It showed two separate pulp chambers in the large central incisor, which were joined together at the cervical area, and one root canal. The lateral incisor presented separate pulp chamber and root canal, but its root canal was connected to the root canal of the large central incisor. These observations suggested gemination of the central incisor fused with the lateral incisor (type IIb of the classification by Shilpa and Nuvvula). |
An 86-year-old female was diagnosed with moderate spinal stenosis in the L2-L5 lumbar spine region. She had a history of chronic LBP and lumbar radicular pain on both sides since 2015. Over the last year, she had been pharmacologically treated with nonsteroidal antiinflammatory drugs and acetaminophen. Her medical history included hypertension and an old cerebrovascular accident, for which she was taking ginko.\nShe had recently complained of severe back pain and radicular symptoms around the distribution of the L5 nerve root. Neurological examination results were normal with a bilateral motor strength of 5/5 in the lower extremities. Her blood tests were all in normal ranges. She agreed to stop taking ginko during her visit for treatment. She laid prone and a pillow was placed under her lower abdomen. The TFESI was performed under fluoroscopic guidance, with a 22-G Tuohy epidural needle at both intervertebral foramen between the L5 and S1 levels. After penetrating the skin, there was no evidence of intravascular uptake or intrathecal distribution. We injected 3 ml of contrast on both sides, but the anterior spreading did not work well, so we decided to inject another contrast to check anterior spreading. As soon as the contrast was injected to the left, she complained of extreme pain and the fluoroscopy image showed the contrast spreading well. The lateral view showed the contrast to be anterior spreading ().\nDuring recovery, she continuously complained of pain and an inability to move. For pain relief, 25 mg of intravenous pethidine was administered, but the pain did not improve. An MRI scan was performed within three hours which revealed a large amount of epidural and subdural hematoma at the lower T-L spines and sacrum with cord compression at the T spine ().\nPhysical examination showed that lower extremity motor function was a grade 5/5 and anal sphincter tone was normal. Under fluoroscopy-guided, the procedure was performed with the patient in a prone position with an 18-G Tuohy needle inserted at the T12-L1 and L3-L4 levels (). After a 20-G multi-orifice epidural catheter was inserted 1 cm upward into the epidural space, approximately 5 ml of diluted watery blood was aspirated from the epidural space. This aspiration occurred three hours after the procedure was performed, so the aspirated blood was significantly more diluted than that of the patient in the first case, and the resistance was not severe. During the procedure, the patient did not complain of any discomfort and her pain decreased significantly after the procedure.\nEmergency surgery was planned in the event that neurological symptoms occurred, but within three days, the patient was able to start walking with reduced pain. A follow-up MRI taken nine days later, the hematoma was shown to be significantly reduced and the patient no longer complained of back pain (). She was discharged from the hospital without any particular complications. |
A 69-year-old male presented to our emergency department with a history of generalized tonic clonic seizures for the last 4-5 years, which have increased in frequency for the last 6 months. He was born out of a nonconsanguineous marriage. Birth history was indicative of a full-term normal delivery without any antenatal or perinatal complications but patient was noticed to have right sided hemiatrophy and hemiparesis at the time of birth but no medical record is available. According to patient siblings despite this right sided weakness, he had normal developmental milestones and was able to manage the activities of his daily living independently but was noticed to have hearing problem in early childhood but no medical attention was sought for any neurological problem so far.He is working as a plumber, has 4 brothers and one sister with no family history of any neurological illness.\nHe started to have generalized tonic clonic seizures around 5 years ago, initially very infrequent, recurring every 6-8 months but became more frequent over the last 6 months with 4 episodes of generalised tonic clonic seizures during the last week that brought him to emergency department. There was no associated history of fever or mental status changes, so no suspicion of central nervous system infection arose. The timeline of patient illness and follow-up is presented in .\nHis examination did not reveal any manifestations of neurocutaneous disorder. His cognition and orientation was intact. He scored 24/30 on mini mental status examination, which was appropriate provided his poor educational background. There was mild left facial atrophy, left ear deafness and could barely hear from his right side but rest of the cranial nerve exam was unremarkable. His motor system exam revealed right sided spastic hemiparesis of grade 4/5 medical research council with hyperreflexia and extensor planter response. His gait was also hemiplegic ().\nAll the baseline metabolic and septic workup turned out to be negative as the cause of his symptoms. Patient underwent computerized topography from the Emergency department. The cranial CT showed sign of volume loss more severe in the left hemisphere manifested by dilatation of the ventricular system and prominent sulci and extra-axial spaces, consistent with left cerebral hemiatrophy. There is also enlargement of the left frontal air sinus with falcine displacement to the right side (). Correlation with the patient’s clinical history of congenital anomaly since birth and facial asymmetry, this might represent Dyke-Davidoff-Masson syndrome. MRI brain was planned but could not be performed as patient had history of some dental braces that was not compatible with the MRI.\nPatient had generalized seizures and was diagnosed with epilepsy, he was started on single antiepileptic tab phenytoin 300 mg at night.\nNo new clinical findings were observed during follow-up examinations, patient remained seizure free when seen in the follow-up clinic after 4 weeks and then 6 months. Patient was encouraged to continue treatment. |
The patient was a 46-year-old woman with a history of breast cancer 3 years earlier. She presented with a pathologic compression fracture in body and both pedicles of the third lumbar vertebra (L3), verified by bone scan and lumbar magnetic resonance imaging (MRI). The patient underwent PV with PMMA on July 27, 2009.\nA vertebral body biopsy prior to cement injection was done, and the procedure was subsequently completed uneventfully using the bilateral transpedicular approach guided by C-arm fluoroscopy.\nWithin the first hour post-procedural period, while the patient was in the recovery room, she complained of abdominal pain, which improved within minutes, followed by severe pain and numbness in the left leg. The left foot of the patient was found to be pale and cold with the sign of developing mottling and cyanosis from the knee level down. The calf muscles were also tender on examination. She had no detectable arterial pulses below her left knee by palpitation, which was confirmed by an emergency Doppler examination revealing the complete lack of blood flow in both dorsalis pedis and posterior tibial arteries.\nConsequently, the patient underwent the computed tomographic (CT) scan of the lower limbs and abdomen, which showed hyperdense foci (cement) in the anatomic site of the popliteal, anterior tibial, posterior tibial, and peroneal arteries as well as in the left L3 lumbar artery extending into the abdominal aorta situated above its bifurcation ().\nThe diagnosis of acute left leg ischemia due to the acute occlusion of the 3 infrapopliteal arteries by cement was considered. A loading dose of 300 mg Aspirin and 450 mg Clopidogrel was given, intravenous (IV) heparin was chosen as a foundation anticoagulant, and lower limb angiography was immediately performed with vascular access obtained in the right femoral artery. The angiogram revealed scattered opacity in the otherwise normal left iliac and superficial femoral arteries. The proximal portions of the popliteal arteries had also acceptable blood flow but thereafter a cement cast existed causing a poor run-off from the popliteal fossa distally ().\nAfter discussing the case with the vascular consultant, since there were diffuse and long vessel involvements and there were no distal targets for by-pass vascular surgery, we opted for transluminal angioplasty (PTA) of the infrapopliteal arteries. We used the crossover approach via the contralateral common femoral artery to access the infrapopliteal arteries. Multilevel intervention was performed through a long 6-F Shuttle sheath (Cook Medical), and total occlusions were traversed with 0.014-inch hydrophilic wires (Shinobi, Cordis, Miami, Florida).\nWe were able to dilate the lesions of the anterior tibial artery at its origin, proximal, and mid parts by utilizing a 2.5 mm × 120.0 mm Pacific Xtrime (Invatec, Rocandelle, BS, Italy) balloon, while the distal portions of this artery and the dorsalis pedis artery were repeatedly inflated with a 2.5 mm × 30 mm Sprinter balloon catheter (Medtronic, Inc., Minneapolis, Minnesota) (). Then in an effort to dilate the totally occluded posterior tibial artery at the bifurcation of tibioperoneal trunk, the balloon was attached to the cement lining in the artery wall, and part of its distal fragment remained in this vessel (). Finally, utilizing a 2.0 mm × 80.0 mm Amphirion Deep (Invatec, Rocandelle, BS, Italy), balloon, angioplasty was performed for the peroneal artery with good results. The final imaging showed an acceptable blood flow in the anterior tibial artery, and in the tibioperoneal artery (). Although finally the occluded parts of the dorsalis pedis and peroneal arteries did not respond to balloon dilation, the flow to the distal bed seemed to have been improved significantly mainly by the collaterals from the proximal and mid parts of the anterior tibial artery and the peroneal arteries. The overall time in the Catheter Laboratory was about 3 hours. The patient’s postoperative course was uncomplicated; the extremity tenderness and mottled skin were improved. An arterial duplex scan performed on postoperative day 5 showed normal velocities with mild stenosis in the1/3 distal portions of the anterior and posterior tibial arteries. Also, no flow was observed in the dorsalis pedis artery. She was discharged on postoperative day 10 on Clopidogrel (75 mg/day) and Aspirin (80 mg/day). A follow-up ultrasound 2 months later revealed an acceptable distal flow in the arteries of the affected limb, and the patient remained asymptomatic (except for a mild leg pain on exertion) at the 1-year follow-up examination. |
A 62-year-old Asian male presented to an outside institution with chief complaints of epigastric abdominal pain and reflux symptoms. Review of systems, past medical history, physical exam, and laboratory values were unremarkable. Family history was notable for pancreatic cancer in his father at the age of 92 years. An upper gastrointestinal contrast study was obtained and revealed a 6 cm mass within the duodenum that resulted in significant compromise of the lumen. A computed tomography (CT) scan demonstrated a cystic and solid lesion located within the duodenum and impinging on the head of the pancreas. Esophagoduodensocopy (EGD) and endoscopic ultrasound (EUS) demonstrated a submucosal, cystic lesion in the wall of the duodenum distal to the ampulla of Vater. The patient underwent an endoscopic ultrasound with multiple biopsies and fluid aspirations. Microscopic evaluation revealed benign glandular cells with reactive changes. No malignant cells were identified. Endoscopic un-roofing of the cystic lesion was performed. Clear viscous fluid was noted to emanate from the lesion and again pathology demonstrated only benign glandular cells with reactive changes. Despite this procedure the mass was noted to recur and grow in size over the next three 3 years. Over this time the patient did not experience vomiting or weight loss but did have significant worsening of his reflux symptoms.\nThe patient was referred to our institution and evaluated by a multidisciplinary gastrointestinal oncology team. CT imaging at that time demonstrated a massive intraluminal mass extending from the antrum through the duodenum (Figure ). Based on this finding and previous failed attempts at endoscopic management it was decided that this tumor could not be resected endoscopically. He was offered surgical exploration and resection. Preoperatively, it was felt that this lesion could be removed through a trans-duodenal local resection. At operation the tumor was found to have a broad-based attachment to the duodenal wall and a local excision was not possible (Figure ). The patient underwent a pancreaticoduodenectomy. Surgical reconstruction was performed with a Peng end-to-end binding pancreaticojejunostomy as previously described [] with the exception of placement of a 3.5 French plastic pediatric feeding tube as a pancreatic stent []. Three 10-mm Jackson-Pratt silicone drains were left at the pancreaticojejunostomy and hepaticojejunostomy anastomoses as previously described []. The patient advanced to a regular diet by postoperative day (POD) 6 but had amylase-rich drain output of less than 200 milliliters per day. As a result of the high-output postoperative pancreatic fistula, the patient was maintained on a low-fat diet and discharged home POD 19 with the drain that was removed in clinic POD 34.\nPathologic examination demonstrated a Brunner's gland hamartoma measuring 10.5 cm (Figure ). No dysplasia or malignancy was seen within the entirety of the specimen. The lesion was composed of back to back mature Brunner's glands. |
Two 27 year-old male MZ twins were referred to our Unit for molecular analysis of the NF1 gene. The probands were born after an uncomplicated pregnancy to healthy non-consanguineous unaffected parents, with no prior family history of NF1. The twins have an unaffected brother 4-years older who suffered mild psychomotor delay and dysphasia in early childhood. The twins grew to adulthood and they are currently still living together.\nBoth twins presented with minimal cutaneous manifestations, including approximately 15 CLS and a few cutaneous neurofibromas (detailed clinical data is reported in Table ). A relevant tumour phenotype was observed in both twins. In "Twin 1", MRI performed at 20 years of age detected one PNF of the left sciatic popliteal nerve and many small neurofibromas in both legs surrounding the femoropopliteous vessels. Although the PNF was resected due to the distal pain in the leg, at 22 years of age a further surgical intervention was performed to resect a tumour detected by MRI in the same region. Pathological analysis of the resected tissue diagnosed a MPNST of the sciatic popliteal nerve and a PNF. Although the patient was subjected to a cycle of radiotherapy after surgery, the MPNST recurred twice 2 and 4 years later. These tumours were treated surgically, leading to the amputation of the left leg in the last operation. In "Twin 2", MRI examination at 20 years of age identified bilateral PNFs of the sciatic nerves and at 24 years of age, the tumour in the left leg was surgically resected due to its rapid growth and malignant radiological aspect. The pathological analysis of the resected tissue diagnosed high grade MPNST of the left sciatic nerve and hence, a cycle of radiotherapy was administered after surgery.\nA pulmonary metastatic MPNST was detected in both twins at 27 years of age. "Twin 1" developed the metastasis in the lower lobule of the right lung, while "Twin 2" presented the metastasis in the upper lobe of the left lung. The concordant and discordant manifestations associated with NF1 in these twins are presented in Table .\nLymphocyte RNA was obtained from fresh peripheral blood and RT-PCR was performed to amplify overlapping cDNA fragments that covered the entire gene. The PCR products were analyzed by Denaturing High Performance Liquid Chromatography (DHPLC) and the positive fragments in the DHPLC analysis were sequenced. The mutations found were confirmed by direct sequencing of the genomic DNA and in this way, the c.4537 C→T mutation (Arg1513X) was found in exon 27a of the NF1 gene from both twins. The mutation was not present in the twins' parents or brother.\nTwins' zygosity was confirmed by analysing 14 polymorphic microsatellite markers at different chromosomal loci. Informed consent for genetic analysis was obtained from all family members included in this study. |
This is a case of a 38-year-old man who presented to psychiatry as a referral for depression by neurology. He had been followed by the neurology department for intractable migraine resistant to all treatment for the last year. His past medical history was also significant for obstructive sleep apnea and obesity. The patient arrived at our clinic with dark sunglasses and was accompanied by his mother. He reported that he had had headaches for the last year with no relief. He denied any history of prior headaches. Imaging was performed to look for an organic cause of the headache and rule out structural deformities. A CT of the head without contrast revealed a nonspecific deep subcutaneous soft tissue mass overlying the left occipital bone, measuring approximately 2 cm at the basal diameter. An MRI of the brain without contrast showed small vessel changes. Under the care of neurology, the patient tried sumatriptan, rizatriptan, topiramate, propranolol, nortriptyline, trazodone, and valproic acid and had recently undergone Botox treatment with minimal relief. He rated the pain an eight out of ten in the moment and a ten out of ten at its worst. The pain was frontal and located over his left eye. This was debilitating him to an extent where he had to resign from his job last year as a truck driver; since then he had been homebound. He preferred to stay in his darkened room all day. He stated that people talking, bright lights, and outside noise made his pain worse; therefore, he had no interest in going out and seeing people. He reported sleep lasted only three to four hours a night, but since he received his sleep apnea machine a couple of weeks ago, his sleep increased to four to seven hours a night. However, his sleep continued to be disturbed, with ongoing headaches, and he lacked energy when he woke up from sleep. He also developed poor appetite, hopelessness, depressed mood, and passive suicidal ideation. These feelings had gradually worsened in the past year. He denied suicide attempts or inpatient psychiatric admissions in the last year. He was seeing an outpatient therapist.\nHis developmental history was significant for perinatal asphyxia. Collateral information was collected from his mother, who described complications at birth, stating that the “cord was wrapped around his neck, and he came out blue.” Growing up, he reported severe irritability, impulsivity, and risk-taking behaviors. According to the mother, when she and her husband got divorced, the patient reacted impulsively by holding a knife to his own throat. He also, at the peak of his impulsivity, tried to hang himself on the neighbor’s tree when he was 10 years old. She states the patient never physically harmed anyone but did push his sister up against a car once for driving too quickly and caused his mother to hit her head. The patient was not evaluated by a child psychiatrist at that time as far as we know.\nHe admitted to homicidal ideation in the past and road rage without physical altercations. The patient also reported having learning difficulties in school, being unable to concentrate, and thus unable to complete his education. He reported that his behavioral issues and mood symptoms suddenly stabilized after he received a warning for his ill-conduct at the age of 23 from a judge. According to the patient, he did well from the age of 23; he led a stable lifestyle as a truck driver, denied road rage, and denied illicit substance use. He denied any hypomanic or depressive mood symptoms until last year when the headaches began. He did not receive any psychiatric care during that period. However, since the headaches began, he was seeing an outpatient therapist who diagnosed him with bipolar disorder. He was prescribed valproic acid 1500 mg daily for his mood symptoms and headaches by neurology.\nOur clinic diagnosed him with an unspecified mood disorder and initially tried prozac for his depression and impulsivity symptoms. The patient did not observe any change in mood symptoms but felt his headaches were worsening on prozac. Considering his history of perinatal asphyxia, small vessel changes seen on MRI, and constant headaches along with mood symptoms, we started a trial of gabapentin. We communicated our concerns to neurology regarding his headaches as likely being secondary to cerebral vasculitis; however, the workup was pending. On gabapentin, the patient had observed his headaches and his mood symptoms to be better; however, he developed extensive bipedal edema. He was unable to walk, so the medication was discontinued. As we continued to explore further options, his mother later reported a history of migraine and mood symptoms in herself that had responded well to topamax. We always collaborated care with a neurologist and discussed the possibility of a trial of topamax. However, before it could be started, the patient’s care had to be transferred to another psychiatrist, and we could not obtain further details concerning his outcome. |
A 45-year-old woman presented to our hospital with multiple lung nodules. She had a history of poorly differentiated thyroid carcinoma, diagnosed 7 months prior to admission, at an outside hospital. The patient was healthy otherwise and reported no radiation exposure or any family history of thyroid cancer. The initial work-up at the time of discovery of the right thyroid nodule included fine needle aspiration and core biopsy, with findings consistent with poorly differentiated thyroid carcinoma. The patient then underwent a total thyroidectomy and central neck lymph node dissection. The pathologic diagnosis from the outside hospital reported a 2.8 × 2.4 × 1.1 cm tumor in the right thyroid without extrathyroidal extension or lymph node metastasis. However, both capsular invasion and extensive vascular space invasion were noted. Based on the tumor size, tumor extension and lymph node status, the tumor was designated as Stage II (pT2 pN0 pMx). IHC staining showed that the tumor cells were positive for thyroglobulin and thyroid transcription factor 1 (TTF1). An immunostain for p53 was also performed at the outside hospital and showed a small focus (< 1 cm) with p53 positivity, suggesting a diagnosis of anaplastic thyroid carcinoma.\nAt our institution, the diagnosis was revised, based on review of both the primary thyroid tumor and the current lung metastases. Both tumors were remarkable for biphasic malignant components: the carcinoma and the sarcoma. The carcinoma component showed a poorly differentiated microfollicular type thyroid carcinoma, composed of sheets and islands of tightly packed thyroid follicles with dense colloid. The tumor nuclei were small and round with vesicular chromatin, resembling those of typical poorly differentiated follicular thyroid carcinoma. Admixed with the epithelial component were malignant spindle cells with small round blue cell type morphology. Focally, rhabdomyosarcoma-like cells with eosinophilic cytoplasm were appreciated. No heterologous cartilage or bone components were identified. The IHC staining performed at the outside hospital showed that the thyroid carcinoma (epithelial) component was positive for thyroglobulin, PAX8 and TTF1 (Fig. ). The sarcoma (spindled) component was negative for all thyroid carcinoma markers (TTF-1, thyroglobulin and PAX8), but was positive for vimentin and focally positive for myogenin (supporting skeletal muscle differentiation) consistent with mesenchymal differentiation. Interestingly, the foci of vascular space invasion contained both epithelial and mesenchymal components as well.\nThe patient received Taxol with Carboplatin for 7 weeks followed by radiation therapy. Her thyroglobulin level rose from 1.2 ng/mL to 25.40 ng/mL 5 months after completion of the chemo-radiation therapy, suggesting progression of the disease. A follow-up CT scan of the chest showed multiple newly developed nodules (ranging from 1 to 2 cm) in the right lung, highly suspicious for metastases. The patient underwent a right thoracotomy, right lung resection/metastasectomy. The surgery was uneventful with negative resection margins. However, the patient’s general condition deteriorated and she succumbed to the disease 4 months later.\nHistological examination of the lung nodules revealed similar tumor morphology and tumor differentiation when compared to the original thyroid tumor, which is somewhat unusual for a biphasic carcinosarcoma (Fig. ). Tumor necrosis was also present. Mutational analysis using a next-generation sequencing based assay showed that the neoplastic cells from the lung metastasis were devoid of genomic alterations for known thyroid cancers, including BRAF, RAS family (KRAS, NRAS and HRAS), EGFR, PTEN, TERT, PI3Kinase or RET. BRAF or RAS family are known as the most commonly altered genes in papillary thyroid cancers. Other molecular mutations reported in the development of anaplastic thyroid carcinoma include p53, PAX8/PPAR gamma rearrangement []. None of the mentioned gene mutations were identified in our patient.\nHowever, an interesting finding in this case is the presence of a point mutation in DICER1 (E1705K) that has previously been associated with differentiated thyroid carcinoma [, ]. Whether the DICER1 (E1705K) mutation is the underlying genetic event leading to the initiation of tumorigenesis or is downstream to other gene alterations in tumor development is largely unknown. Additional mutations of unknown significance were also detected in this tumor including FLCN (R239H), POLD1 (Q684H) and SYK (R217L). These variants have not been adequately characterized in the scientific literature and their prognostic and therapeutic significance is unclear. |
A 34-year-old male was admitted to the hospital with recurrent episodes of retrosternal chest pain, fatigue, and shortness of breath with an elevated troponin T. He had suffered an acute episode of myocarditis four years previously requiring hospital admission. He had no other relevant medical history and no family history of cardiac disease. He is a nonsmoker and consumed alcohol occasionally. Clinical examination was unremarkable and did not show any evidence of heart failure or systemic disease. ECG showed normal sinus rhythm without any ischemic changes, and chest X-ray showed no evidence of infection or heart failure. Routine blood tests including antinuclear antibody, creatinine kinase (CK), rheumatoid factor, and C-reactive protein were all within normal limits apart from an elevated cardiac troponin T with a peak value of 2700 ng/l (<14 ng/l). Further extensive inflammatory, viral, and autoimmune screening was carried out and found to be negative. Subsequent coronary angiogram showed normal coronary arteries, and transthoracic echocardiography demonstrated left ventricular ejection fraction (LVEF) >55% with trace mitral regurgitation. Cardiac magnetic resonance imaging (MRI) demonstrated extensive subepicardial and midwall late enhancement typical of myocarditis in the anterior, lateral, and inferior walls along with extensive fibrosis with normal LVEF ().\nA short course of steroids and anti-inflammatory medication as an inpatient resulted in the resolution of his myocarditis symptoms. The troponin T level normalized and the patient was discharged with a plan to repeat cardiac MRI in six months. On follow-up as an outpatient, it was decided to refer the patient to rheumatology for an opinion regarding ongoing immunomodulatory therapy. At this juncture, the patient stated that he also had symptoms of stiffness and aching in his calf muscles for quite some time but he did not consider it to be relevant. Despite persistently normal skeletal muscle enzyme levels, an MRI of the lower legs was performed and this showed active myositis involving the gastrocnemius muscles bilaterally (). As the patient was demonstrated to have ongoing myositis despite minimal symptoms, and as he had accrued significant myocardial scarring from previous episodes of myocarditis, it was decided to commence long-term immunomodulatory therapy in the form of methotrexate and prednisolone. Clinically, the patient reported a significant improvement in his symptoms and a repeat of the lower limb MRI demonstrated a significant interval improvement in his skeletal muscle myositis. Six months later, a repeat of the cardiac MRI demonstrated resolution of myocarditis along with persistent, stable, and extensive myocardial fibrosis and preserved LVEF (). The patient is tolerating the immunomodulatory therapy well without major side effects, and he has returned to full-time work. |
The patient was a girl who had suffered from six episodes of febrile seizure since 1 year old, one of which lasted for 1 h. We performed EEG four times from 2 to 4 years old, and none revealed epileptic discharges. Developmental history was normal. Her mother had a history of febrile seizure, but all other family members were reportedly free from seizure disorders.\nAt 5 years and 6 months old, she was transported by ambulance and admitted to our hospital due to impairment of consciousness. Her parents reported sudden vomiting that started during a nap while traveling in a car, and repeating several times. She woke from the nap, and was initially able to speak, but gradually became unconscious. Her eyes then deviated to the right, her complexion became pale, and her limbs became flaccid. When the patient arrived at our hospital 70 min after seizure onset, she was still lethargic, but her eyes were closed without deviation. The emergency room doctor decided that the seizure had already stopped and she was in a state of post-ictal drowsiness. EEG revealed epileptic spikes in the left occipital region, whereas magnetic resonance imaging (MRI) showed no abnormalities. PS was then diagnosed. After observing the course without treatment, the patient experienced another seizure 9 months later. This time, the seizure evolved to left hemiconvulsions with eyes deviating to the left. Medication started with valproic acid (VPA), with later addition of clobazam, but neither achieved resolution of seizures. In total, she experienced nine seizure events equivalent to PS by 7 years and 4 months old, most of which were lengthy seizures lasting >5 min or SE >30 min. The longest seizure lasted 60 min before stopping on intravenous injection of diazepam. Although she experienced no further PS seizures after 7 years old, EEG spike discharges were consistently observed. Medication was continued in accordance with the wishes of the parents until 13 years 6 months old, by which time the EEG abnormalities had disappeared.\nAt 14 years 6 months old, the patient noticed twitching in the arms, particularly on waking. That same month, she suffered GTC right after waking. EEG showed a 6-Hz wave-and-spike phantom, and results of MRI were normal. Based on the seizure symptomology and laboratory findings, we diagnosed JME. Levetiracetam (LEV) was started, and completely eliminated seizures without adverse effects. The patient is currently 17 years 7 months old and has shown no further symptoms. |
Patient A was a 62-year-old woman who developed kidney failure in her late teens secondary to mesangiocapillary glomerulonephritis. She had a live donor kidney transplant aged 34, which failed 13-years later and a cadaveric transplant the year after lasting 8 years. Her past medical history included hypertension, acute myocardial infarction, and a cerebrovascular accident. After 5 years of hemodialysis following the failed cadaveric transplant, the idea of withdrawing from active therapy was discussed at a time she described as “the low point in my life.” Psychiatric care through this period had no effect on her functional state. She reiterated her desire to withdraw from therapy because of the combination of physical symptoms of recurrent brachiocephalic thrombosis, nausea, vomiting, and a decline in overall functional status. With her family closely involved, discussions around prognosis and quality of life were raised and a joint decision was made to continue management in a palliative care setting. This family meeting, along with ongoing follow-ups with the renal team reinforced the relationship where Patient A felt that she was supported in her decision to discontinue dialysis therapy. An interdisciplinary approach was facilitated by referrals to social work and pastoral care.\nDuring this period the patient underwent some palliative ultrafiltration sessions, which helped in the resolution of her progressive breathlessness. The patient had a planned admission to a specialist palliative care unit immediately following her complete withdrawal from dialysis. She experienced some episodes of nausea, shortness of breath and agitation, all managed effectively by the palliative care team. Food and drink were provided and consumed as tolerated. She was well supported by her family during her admission and there were no unrealistic expectations of her family with regards to the aims of palliative therapy. She understood and even stated that “I only have a few days of life left” and spent quality time with her loved ones towards the end. The patient passed away in the palliative care unit 10-days after withdrawing from dialysis. |
Patient II: This 40-year-old woman, mother of patient I, reported tremor and mobility problems since early childhood. She could never keep up with her peers in sports activities. She was also found since early childhood to have dental anomalies requiring multiple procedures for correction. Adhesion of digits 4 and 5 bilaterally in the hands were treated surgically in childhood. She reported learning difficulties throughout her school years. She complained about progressive difficulty walking, impaired balance, and urgency of urination with occasional incontinence. Besides her affected daughter, no other affected family members could be confirmed. Her father was seen by the neurologist and did not have any apparent features of the disease. Her mother died at the age of 38 of unknown etiology and it remains unclear whether she had any features of the syndrome. Examination revealed the typical dysmorphic features of ODDD. Her hearing and vision were intact. She has mild dysarthria, which might be related to the nasal dysplasia. On motor examination she has full muscle power throughout, but fine motor movements were impaired in the hands and feet and she had postural and action tremor with mild dysmetria. There was increased muscle tone with spasticity especially in the lower limbs. Reflexes were brisk throughout. Plantar responses were extensor bilaterally. There was no cerebellar dysfunction. Sensory examination showed reduced pinprick and vibratory sensation in distal limbs. Her Romberg’s test was positive.\nFurther investigations in patient II included normal routine hematological and complete biochemistry studies, thyroid function tests, vitamin B12, and folic acid levels. An MRI of the brain, showed evidence of leukodystrophy with delayed myelination of the cerebral white matter (). Characteristic dysplastic changes in the facial bones with hypoplasia of the paranasal sinuses, as well as aplasia of the frontal sinuses associated with thickening of the frontal calvarium were also noted (). MRI of the spine revealed no spinal cord abnormalities but there were mild degenerative disc and arthritic changes causing a mild degree of stenosis of the lumbar canal. Visual evoked potential studies revealed prolonged absolute P100 latencies bilaterally (left 130 ms, right 134 ms) indicative of optic pathway dysfunction. Brainstem auditory evoked potentials revealed prolonged absolute wave V and I–V and III–V interpeak latencies bilaterally at 80 dB nHL, indicative of bilateral acoustic pathway dysfunction between the pons and midbrain. Finally, both upper as well as lower limb somatosensory evoked potentials revealed prolonged latencies corresponding to somatosensory pathway dysfunction throughout the CNS. A routine EEG revealed no abnormalities. EMG/nerve conduction studies showed no evidence of peripheral neuropathy, but there was evidence of mild chronic right L4–L5 radiculopathies. An echocardiogram revealed no abnormalities and a 24-h 3-Lead Holter monitoring study showed normal sinus rhythm with multiple episodes of sinus tachycardia up to 140/min. |
A 62 year old woman sustained a closed fracture of right humeral shaft resulting from a car accident (OTA 12A3). We performed the open reduction and internal fixation through an anterolateral approach under general anesthesia and placed a 4.5 mm dynamic compression plate on the lateral aspect of the humerus. The placement of the implants was good except that the tip of the most proximal screw protruding approximately 1 cm through the medial cortex []. During in-hospital treatment, the patient recovered without complaining any discomfort of the right hand. 4 weeks postoperatively, however, she began to feel tingling over the thumb, index and middle fingers of the right hand, which was worse at night. The symptoms gradually progressed with thumb-index finger pinch weakness. When coming to our clinics 3 months postoperatively, she presented a sensory deficit of those fingers. Both Tinel's sign over carpal tunnel and Phalen's test were positive, as well as atrophy of the thenar muscles. She did not experience such symptoms prior to surgery to the humerus fracture. The neurophysiological study confirmed the clinical suspicion of CTS, with a prolonged distal latency and low amplitude of compound muscle action potential, so did the sensory nerve []. The neurophysiological finding of the proximal median nerve and ipsilateral ulnar nerve was normal. The criteria for electro-diagnosis of CTS included: Distal median motor latency >4.4 ms; difference between distal motor latency of median and ulnar nerve >1.1 ms; difference between distal sensory latency of median and ulnar nerve >0.2 ms. Although a transient improvement was observed with night splint treatment, the symptoms recurred after a few weeks. A carpal tunnel decompression was therefore recommended but the patient refused.\nAt 16 months postoperatively, the patient asked for removal of the implants. During the operation, we observed that the tip of the protruding screw twisted the median nerve with scar tissues. Extensive neurolysis around the median nerve was not performed, because we did not expect relief of symptoms of CTS after removal of the implants. At 2 weeks later, however, the patient experienced a significant relief of the tingling over fingers. At last review, 6 months after the implants removal, her sensations returned to near normal with full movement of the fingers. Although mild thenar atrophy was still present, the patient remained asymptomatic and denied additional carpal tunnel decompression surgery as she had no great difficulty in carrying out her daily activities. |
A 34-year-old Caucasian female, who had originally undergone an uneventful laparoscopic adjustable gastric band 4 years ago, presented to the bariatric surgery clinic with inability to tolerate solids. A work-up revealed that the laparoscopic band remained in good position but the patient had oesophagitis and gastritis, causing swelling of the mucosa at the band site. The fluid was removed from the reservoir, and the patient was treated conservatively with anti-reflux medication and a full liquid diet. After 2 weeks of treatment the patient’s symptoms improved. After careful consideration, she wished to undergo revisional surgery converting the laparoscopic adjustable gastric band to a laparoscopic vertical sleeve gastrectomy. The patient moved through the appropriate multidisciplinary team approach and was found to be an appropriate candidate for surgery. She underwent laparoscopic removal of the adjustable gastric band and conversion to a laparoscopic vertical sleeve gastrectomy without complications. Her post-operative course was uncomplicated and she was discharged on post-operative day 3.\nOn post-operative day 12, the patient was readmitted to an outside tertiary care hospital for lightheadedness and shortness of breath and was found to have leukocytosis, with white blood cell count of 18,000 cells μl–1. The work-up included a CT scan with intravenous contrast of the chest, abdomen and pelvis, and the patient was diagnosed with a pulmonary embolism. The patient was immediately transferred to our centre for definitive care. When the patient arrived at our centre, the CT films from the outside hospital were reviewed by our radiologists and there was concern that there was air and a faint suggestion of oral contrast outside of the suture line (). Given this finding, an UGI evaluation was ordered. During the early phase, no leak was observed, owing, in part, to the slow passage of 30 ml oral non-ionic contrast (). Some residual contrast from the outside hospital CT was present in the transverse and descending colon. Only after delayed imaging and with administration of additional non-ionic contrast for a total of about 65 ml (approximately 2 h after the start of the fluoroscopic examination) was there a faint suggestion of extravasated contrast, best seen below the left hemidiaphragm (). Follow-up CT scan with oral contrast confirmed the obvious leak ().\nThe patient was treated definitively with endoscopic stent placement and clipping using an Ovesco clip (Ovesco Endoscopy AG, Tubingen, Germany) to close the leak. After an extended hospital course, she was discharged and is presently doing well. |
A 55-year-old female who was referred from the oncology department complained of multiple asymptomatic reddish skin nodules at the left side of the neck of 3-week duration.\nThis condition started 6 months before when the patient was admitted because of jaundice and general fatigue accompanied by multiple enlarged firm, nontender left cervical lymph nodes. Laboratory tests showed raised both total and direct bilirubin, raised liver enzymes; hepatitis markers were negative and renal function tests were normal. Chest X-ray was free; abdominal ultrasound showed a mass located at the head of pancreas measuring approximately 4.6 × 4.8 cm (AP × W) with multiple enlarged porta hepatis lymph nodes with evidence of dilated intrahepatic biliary radicals and dilated common bile duct. A computerized tomography scan (CT) of the abdomen revealed the enlarged head of pancreas with heterogeneous soft tissue mass measuring 5 × 5 cm with multiple porta hepatis and para-aortic lymph node enlargements and no evidence of hepatic focal lesions (). Metastases elsewhere were not detected by examination and thorough investigations. Abdominal ultrasound (US) and CT findings were compatible with a cancer of the head of pancreas with multiple metastatic abdominal lymph nodes causing common bile duct obstruction. On abdominal exploration, cholecystojejunostomy and enteroenterostomy were performed; however, the surgeons refused to take a biopsy from the unresectable mass because of the fear of complications arising from a pancreatic fistula.\nOur patient started palliative cytotoxic treatment. During treatment, she developed asymptomatic violaceous nodules and indurated plaques over the skin on the left side of the neck and she was referred to the dermatology department for consultation (). There were no other similar lesions elsewhere on the body. A lymph node biopsy revealed metastatic carcinoma and skin biopsy revealed nests of poorly differentiated atypical cells throughout the dermis (). Silver stain and chromogranin were negative, while EMA was reactive for tumor cells and CA 19-9 was focally positive (). In light of the patient's history of a cancer head of pancreas and the positive immunohistochemical stain result with CA 19-9 for skin biopsy, the diagnosis of a metastatic pancreatic carcinoma was established.\nOne month later, while receiving the palliative cytotoxic treatment, the reddish, nontender indurated plaques increased in size covering the left side of the neck (). At that time, a follow up CT demonstrated a decrease in the size of the pancreatic mass that reached a craniocaudal diameter of 4 cm. |
A 23-year-old French man of African origin, an elite football player, sustained a midshaft anterior cortex tibial stress fracture 2.5 years ago. Initially, he was treated with cast immobilization, no weight bearing for 3 months, ultrasound stimulation, and electromagnetic field therapy. The fracture did not heal; he had pain during gait, so he continued no weight bearing for 3 additional months. After that period, the fracture site still was not healed, so he underwent an operation performed by his team doctor. In this operation, the medullary canal of his tibia was reamed and an im nail was inserted.\nUnfortunately, the fracture site did not consolidate again, even 18-months postoperatively, so he presented to our clinic for counseling. It was obvious from the X-ray (Fig. ) that a nonunion of the fracture had occurred.\nHe did not smoke tobacco and he had a free medical history. When he presented to our clinic, the area at the fracture site was swollen and painful when palpated. The pain got worse when he attempted to walk with full weight bearing, so he had to use crutches. An examination of the peripheral nervous system of his lower extremities did not provide us with any pathologic findings. In addition, the laboratory examinations for possible endocrine or metabolic disorders were negative (Table ), so he was advised to have a reoperation to address this nonunion. The treatment options for such cases include nail exchange, drilling of the fracture site, bone grafting, or removal of the nail and internal fixation with a plate. We performed a tension band plate fixation, which is a technique already described for the treatment of anterior tibial stress fractures that failed non-operative treatment [], with bone grafting and without removing the nail.\nA longitudinal incision was made just lateral to the anterior tibial crest centered over the fracture site. The fascia over the tibialis anterior was divided, the muscle lifted off and the fracture site was visualized. The necrotic bone and callus at the fracture site was debrided with the use of an osteotome and a curette. Transverse drilling around the fracture site was done to promote healing and osteoblastic activity. Bone marrow from the ipsilateral iliac crest was inserted into the fracture site and a tension band plate was applied over the im nail.\nWe used a 6-hole, 4.5 mm locking compression plate. The plate was prebended and the screws were placed in a compression manner to achieve a tension band effect to the fracture site. A cortical screw was put first to the distal hole closest to the fracture site and then a cortical screw to the closest hole proximal to the fracture site to ensure compression of the fracture. Consequently, one unicortical locking screw was inserted proximally to the fracture site and the other two distally. With the use of locking and non-locking screws we minimized the pressure at the periosteum, which can damage blood supply to the poorly vascularized bone. The screws were angled in a different axis in order to bypass the nail (Fig. ).\nPostoperatively, our patient was advised to wear an orthotic boot and to not bear weight for 6 weeks. Range of motion exercise involving knee and ankle and isometric exercises were initiated immediately postoperatively. After 6 weeks he progressed to weight bearing as tolerated. At 3 months postoperatively he was pain free and started light jogging, swimming, and plyometric and core stabilization exercises. At 6 months postoperatively the complete radiologic union of the fracture was evident (Figs. and ). He was symptom free; he resumed at that time a full training program and he returned to play football 6 months postoperatively at his preinjury high competition level. |
A 69-year-old Filipino man with history significant for hypertension and hyperlipidemia presented to his primary care physician with hematuria with weight loss of 1 month’s duration. He did not have any flank pain, burning on urination, or increased urinary frequency. He did not endorse any symptoms of fatigue or night sweats. His only medication was atenolol for his hypertension. He did not smoke tobacco, drink alcohol, or do any recreational drugs. He was unemployed at time of interview. He did not have any family history of cancer. His vital signs were within normal limits. On physical examination, he was well appearing and in no acute distress. He had no palpable mass and had an otherwise normal cardiovascular, respiratory, and neurologic examination. Laboratory work showed normal cell counts and normal electrolytes; the results of his kidney and liver function tests were normal. A computed tomography (CT) – intravenous pyelogram was performed as a diagnostic work-up for his hematuria, which demonstrated a large mass in the left collecting system and proximal ureter. He was seen by urology with plans for surgical resection 1 month later. Three weeks later he was admitted to the Emergency Department with nausea and vomiting. He was tachycardic to 110 beats per minute but maintained a normal blood pressure. His laboratory results were notable for hemoglobin to 12.1. His sodium was 134. At that time, a CT scan of his abdomen and pelvis showed interval enlargement of the left renal mass. An ureteroscopy with biopsy was performed, which showed necrotic tissue with rare crushed degenerating atypical cells. A screening chest CT scan was also obtained which showed a small 3 mm nodule in the lower lobe of his left lung. A follow-up interventional radiology-guided left kidney biopsy showed a cellular neoplasm with sheets of pleomorphic round cells with hyperchromatic nuclei, irregular nuclear outlines, and inconspicuous nucleoli with scant and delicate cytoplasm which is consistent with SCC. The tumor cells were positive for the neuroendocrine markers synaptophysin and CD56 with focal staining for chromogranin and dot-like positive staining for cytokeratin (AE1/AE3), supporting the diagnosis of SCC (Fig. ). A bone scan did not show any metastatic lesions. Shortly afterwards, he developed dizziness and an MRI of his brain was obtained revealing a 1.6 cm partially hemorrhagic round mass with surrounding edema in the midline superior vermis potentially representing metastatic disease. An additional 4–5 mm hemorrhagic metastatic focus was seen in the right occipital convexity. The cerebellar mass was resected and probably represented a renal origin due to the absence of lung masses along with clinical and radiographic correlation. He was started on whole brain radiation therapy during his in-patient stay. An out-patient oncology referral was made but he was unable to establish care due to frequent hospitalizations. He had several hospital admissions for nausea and vomiting and continued to decline functionally. He developed chronic hyponatremia during these hospitalizations which were attributed to SIADH. He originally presented with sodium of 119 and was stabilized to a sodium level of 128 with the use of salt tablets. He declined chemotherapy when it was offered by the oncology team during in-patient consultation due to poor quality of life and functional status; he died within 8 months of presentation at his nursing facility. The cause of his death was unknown. An autopsy was not performed. |
A 16-year-old male presented with a right abdominal and groin pain. There was no gross hematuria, fever, or lower urinary tract symptoms. Palpation of his right flank area was normal. He had hypertension for 2 years and his blood pressure (BP) measured 190/100 mm Hg at presentation. He had been taking antihypertensive drugs including valsartan hydrochlorothiazide (160/12.5 mg capsule once daily). No other abnormalities were found in the patient's history and on physical examination. Family screening was performed and no abnormalities were found. There was no family history of renal failure or cerebrovascular accident. His chest radiography and electrocardiography findings, urinanalysis, blood urea nitrogen, and serum creatinine levels were within normal limits. Other laboratory tests including lipid profile and liver functions were with in the normal range. His body mass index (BMI) was also in normal range (BMI: 22.1).\nAn abdominal ultrasound showed multiple variable-sized cysts in the upper pole of the right kidney with normal left kidney and lower pole of the right kidney []. Renal arterial color Doppler ultrasonography was normal. Abdominal computed tomography confirmed the ultrasound findings that the upper pole of the right kidney was filled with multiple cysts of different sizes with enhancing normal renal tissue between the cysts. There were no cysts in the other intra-abdominal organs. A renin blood test showed that the plasma renin level was moderately elevated (46 pg/ml, normal limits: 11-33 pg/ml). Selective intrarenal catheterization was performed to obtain venous blood samples. His drug was withdrawn four days before the selective renal vein studies and he was given 10 mg furasemide orally in the afternoon prior to the study. The procedure was started after placing the patient in a supine position for at least two hours. A catheter was introduced through the femoral vein and it was advanced using fluoroscopic guidance. Blood samples for plasma renin level were drawn from both main renal veins and from the upper and lower segmental draining veins of each kidney. The highest renin level was measured in the right upper segmental vein (92 pg/ml). Renin levels of the right lower segmental vein and the left renal veins are shown on .\nIn order to further characterize the lesions, MRI was performed. A gadolinium-enhanced MRI revealed that the upper pole of the right kidney was completely filled with multiple round, well-marginated cysts of varying size without capsule formation []. There were no solid areas within the cysts and renal parenchyma between the cysts was normally enhancing. Technetium-99m-dimercaptosuccinic acid (Tc-99m DMSA) renal scintigraphy showed decreased renal uptake at the upper half of the right kidney. After discussion with the physicians and the parents, a decision was made to perform partial nephrectomy to eliminate the source of the apparent renin-dependant hpertension. The patient underwent right upper pole heminephrectomy with a histopathological result confirming LCDK. Nephrectomy specimen on macroscopic examination showed conglomerate cysts of various sizes in the kidney []. Microscopy examination showed cysts containing dark brown or clear serous fluid and surrounded by mono-layered flat-cuboidal epithelium without evidence of tumor or papillary formation [].\nAfter surgery the patient's blood pressure returned to normal levels without any need for antihypertensive medication. The patient is under follow-up on outpatient basis and has maintained good renal function during the last two years. Follow-up ultrasound examinations showed normally appearing residual right kidney and left kidney. |
A 23-year-old male patient presented to us in June 2011 with a deformity of the back, weakness of both lower limbs, and with a history of multiple surgeries on the back. At three years of age, spontaneous disappearance of the right clavicle and upper humerus was noticed without any preceding trauma. Biopsy was taken from the lesion and diagnosis of Gorham's disease was made based on the histopathological examination of the tissue. The disease was subsequently not progressive. In May 2010, he noticed progressive deformity of the upper back, spasticity, and an altered gait. He was diagnosed with myelopathy and kyphotic deformity of the spine. On August ninth 2010 he pedicle screw instrumentation with decompression of the cord. The patient experienced an uneventful post-op period. His spasticity reduced and his gait improved over a period of two months and he was then able to walk normally. On May 25th 2011, a routine follow up X-ray showed breaking of the implant for which a revision surgery was performed the same day. Post-surgically, the patient developed complete weakness of both lower limbs with loss of bladder and bowel control. He was taken for immediate decompression. As the patient's neurology did not improve, a revision decompression was performed on 3 days later. The patient noticed the return of some movement in his right lower limb a few days after surgery. His bladder control returned one month later. He was referred to us for rehabilitation (, ).\nOn examination, the patient showed dyspnoea, tachypnoea, and paradoxical breathing. Neurological examination revealed complete absence of motor power on the left lower limb and the power in the right lower limb was 1-2/5. Sensation was decreased from T5 below. Deep tendon reflexes were exaggerated with ankle clonus and extensor plantars. A loss of the rounded contour was observed on the right shoulder with painless exaggerated passive movements. A chest X-ray revealed pleural effusion on the left side. Chylous looking fluid was aspirated from the left side of the chest and on examination showed lymphocytes. The patient was booked for a thoracic duct ligation. He was also given the option of radiotherapy and pleural ablation. The patient refused any of these treatments. Since the patient's condition remained stable and the osteolysis was not progressing, he was kept under observation.\nAfter 8 months of rehabilitation, the motor power in the lower limbs gradually improved and he was able to stand and walk in parallel bars with the help of left Anklefoot Orthrosisand bilateral knee gaiters. The disease did not show any progression clinically. |
A 52-year old obese man with known ischemic heart disease but no history of any neurological disease underwent coronary artery bypass surgery. Preoperative routinely performed diagnostic workup revealed no significant findings. During surgery he was laid supine on the operating table with both arms fully adducted to his side, fixed in the neutral position. Intraoperative monitoring included electrocardiography, pulse oxymetry and automatic blood pressure monitoring using a standard-size adult cuff affixed to the patient's right upper arm. No particular events occurred during anesthesia or surgery and recovery was good so that patient was transferred within a day from the intensive care unit to the normal ward.\nHowever, on the first postoperative day he complained of wrist drop on the left. Neurological examination revealed a severe decrease in muscle power of the wrist and finger extensor muscles (0/5 MRC) and a slight brachioradialis paresis (4/5 MRC) accompanied by hypoaesthesia on the radial aspect of the dorsum mani. Biceps and triceps reflexes on the affected left arm were normoactive whereas the brachioradialis reflex was diminished. All muscles innervated from the median and ulnar nerve, as well as all the muscles above the elbow remained unaffected. The clinical diagnosis of radial nerve injury was set and rehabilitation therapy was recommended.\nAfter hospital discharge and about three weeks after surgery the patient was referred for neurophysiological evaluation. In the meanwhile the extensor muscles had already begun to improve. Nerve conduction studies of both radial nerves were performed using surface electrodes. Compound muscle action potentials (CMAP) were recorded from the extensor digitorum communis muscle. The opposite radial nerve was examined for comparison. Supramaximal nerve stimulation was achieved by gradually increasing the stimulation power until the point where the amplitude of the waveform did no longer increased was reached. Electrical stimulation at the elbow, below and above the spiral groove, revealed an amplitude decline of the CMAP that was indicative of a partial conduction block of the left radial nerve along the spiral groove, whereas CMAP recordings of the right radial nerve were normal (Table ). Moreover, motor axonal loss due to wallerian degeneration distal to the site of the lesion was suggested by the low distal CMAP. Needle electromyography enhanced this finding by revealing moderate impairment in recruitment with fibrillation potentials in radial innervated muscles below the elbow and normal findings in both triceps and deltoid muscles. The motor unit potentials were normal, a finding that is consistent with a recent nerve injury. In conclusion, all electrophysiological findings were indicative of a radial nerve injury in the spiral groove. The involvement of the brachioradialis muscle and the fact that both deltoid and triceps muscles remained unaffected practically excluded the differential diagnostic alternative of a posterior interosseus neuropathy and a posterior cord brachial plexus lesion respectively. |
The patient was a 67-year-old African American male who had a psychiatric history of schizophrenia as well as a history of benign prostatic hyperplasia and essential hypertension. He had been receiving care for over 10 years at our ambulatory psychiatric center while on a monthly intramuscular injection of 50 mg of haloperidol decanoate. He had a past history of muscle stiffness from the haloperidol injection and this extrapyramidal symptom had been controlled with oral benztropine at 0.5 mg two times a day on an as-needed basis. During the course of his treatment, he developed hematochezia along with lower abdominal pain, constipation, and difficulty with urination, which was initially attributed to his enlarged prostate. However, a careful history by his psychiatrist subsequently revealed that he had been using Benztropine more than he was being prescribed. He admitted to taking more than double his prescribed dose per day as well as increasing doses over a period of months, as he had also been receiving this prescription from his gastroenterologist (up to a total of 5-8 mg per day in divided doses). He did not give any clear reason for taking higher doses of the benztropine and only stated that he was just taking his "side effect medication" as needed. The psychiatrist contacted his outpatient gastroenterologist and urologist, coordinating his care with these specialists. Extensive psychoeducation, over a period of multiple clinic visits, was provided to him focusing on his increased susceptibility to the anticholinergic effects of benztropine. It was explained that his advanced age and history of benign prostatic hyperplasia placed him at an elevated risk of anticholinergic complications. The benztropine dose was reduced and shorter duration prescriptions were provided to allow for increased monitoring and he began to use the medication as prescribed. His gastroenterologist and urologist had both ruled out a malignant neoplasm as the etiology of his presentation. These symptoms gradually resolved and he continued to receive psychiatric care regularly at our clinic without any further incidents. |
A 32 year old lady presented with recent onset left flank pain which was not associated with any precipitating events. She had chronic lower back pain. She had no gross hematuria, fever or lower urinary tract symptoms. Her menstrual history was normal. She reported no abdominal or pelvic pain, no dyspareunia and had normal bowl motion. She gave no history of smoking, past medical surgical or gynecological treatment or intervention. Examination of the patient revealed no abdominal or pelvic tenderness or masses. Urine analyis showed microscopic hematuria and pyuria. Urine culture was positive for Klebsielae pneumoniae. Serum creatinine was 59 umol/L. CT scan without contrast showed no stones and mild left hydronephrosis. The patient was discharged on antibiotics and analgesics for follow up. A 7 day course of ciprofloxacin eradicated the infection. Imaging for evaluation of the lower back pain indicated osteoarthritis at vertebra L5-S1. A bone scan confirmed this diagnosis and accidentally revealed poor tracer uptake of the left kidney. The patient was referred to Urology.\nAn intravenous urography showed no excretion from the left kidney []. A diuretic renogram using 99mTc-MAG3 showed poor excretion of the left kidney []. A retrograde study was carried out which revealed a short narrow segment in the ureter at the junction of the lumbar and pelvic ureter []. Ureteroscopy showed a papillary growth obstructing the lumen of the ureter. A cold cup biopsy was taken from the lesion. A double J stent was left indwelling. Microscopic pathology was not conclusive indicating an inflammatory process. A repeat renal scan with the JJ stent indwelling for 16 days, failed to show any improvement in renal function or excretion []. The patient was informed that in view of the poor functioning kidney and the high risk of harboring a malignant ureteric tumor surgery is indicated. A robotic nephroureterctomy with a bladder cuff was carried out with an uneventful course. During the surgery the left ureter was seen at the pelvic brim surrounded by adhesions and scar tissue. No other gross lesions were identified intraoperatively. Macroscopic examination of the ureter at the site of narrowing showed a firm nodule covered by bluish-red mucosa measuring 1.5 × 1.5 cm. Microscopic examination of the ureteric nodule revealed benign endometrial gland and stroma underlining the normal urothelium [Figure –]. Immunohistochemical studies were performed for estrogen receptor and CD10. The endometrial glands were positive for Estrogen receptor [] while the endometrial stroma was positive for CD10 []. |
A 62-year-old man presented at the Neurology consultation with a six-week history of a severe, strictly left orbitotemporal headache, with a frequency of three attacks per week, occasionally more than one at the same day. Most of them occurred in the first half of the night, waking him up, and lasted between thirty minutes and one hour. He used to take ibuprofen as acute treatment, with unsatisfactory response, since he did not notice a significant difference between treated and untreated attacks in terms of duration and pain intensity. To relief the pain, he used to open the window to get some fresh air. The headache was always associated with ipsilateral conjunctival injection and lacrimation. Pain triggers were not identified by the patient. He had no personal or familial history of headaches. His medical history was remarkable for hypertension and asthma, with a past surgical history including septoplasty and bilateral middle turbinectomy and uncinectomy due to nasal respiratory insufficiency. By the time of medical evaluation he was asymptomatic and neurological exploration was unremarkable. The clinical picture was suggestive of a CH and the patient was medicated with verapamil 120 mg daily. A MRI scan was performed, which revealed a sphenoid sinus mucocele, without secure expansion of the sinus. Two weeks later the patient came to the Emergency Department with complaints of horizontal diplopia that he noted when he woke up in that morning. He maintained the headache attacks, with similar characteristics, despite prophylactic therapy. Neurological examination revealed left eye adduction palsy and ptosis. A brain CT scan was performed and excluded lesions other than the mucocele. Paranasal sinus MRI revealed molding of the medial wall of left cavernous sinus by the sphenoid mass (). A paranasal sinus CT scan was also performed to allow for a better characterization of the lesion, showing sclerosis and interruption of the roof and posterior wall of the left sphenoid hemisinus (). The patient was submitted to surgical drainage of the mucocele by transnasal-transphenoidal approach, with complete resolution of the adduction impairment, persisting a mild left eye ptosis. After the surgery the attacks stopped, and in the six-month follow-up he reported no further attacks. |
A 19-year-old female reported to the casualty department of Vardhman Mahavir Medical College and Safdarjung hospital, New Delhi (a tertiary care university teaching hospital) with the history of cut throat injury occurring about 4 hours back. The patient was brought by the police, as it was referred from an adjoining district hospital with victim's action being reported as a suspected suicidal attempt. On examination, patients vitals were stable. A vertical wound of approximately 8 cm in linear length with clean margins and ooze of blood was seen almost in the middle of the neck. The injury site was having a tracheostomy tube of size: 8.5, through which the patient was maintaining adequate respiration. The referral record indicated that the upper airway had been totally transected and a tracheostomy tube had been inserted in the distal part of the trachea as a first aid measure in the district hospital. No bruises or any other lacerations were seen in relation to the said wound. Also, the hyoid and thyroid cartilage were clinically intact.\nAfter orthopedic clearance for any suspected spinal trauma, the patient was transferred to ENT department and an immediate exploration of the injury under general anesthesia was undertaken. On exploration of the wound, complete cricotracheal transection with wide separation of the ends, anterior wall perforation of the esophagus, and cutting off the strap muscles was observed (). No injury to great vessels or to any other adjoining structures in the neck was observed. A Ryle's tube was immediately inserted and the anterior wall of the esophagus was repaired surgically by suturing. The distal end of the trachea was retracted approximately 3 cm, the trachea was retrieved manually and primary end to end anastomosis of the tracheal injury was done (). The recurrent laryngeal nerve on either side of neck was not looked for. Finally, wound was stitched in two layers with a simple corrugated drain in situ.\nPostoperatively, the patient was put on antibiotics and the drain was removed after 48 hours. The patient underwent CT-Scan (to document the injury for medico legal purposes) and fibro-optic laryngoscopy on the 7th postoperative day which revealed the vocal cords in paramedian position. A barium swallow was done at the end of 2nd week, which was found to be normal, thereafter feeding to the patient was started orally (). A repeat bronchscopy was done at the end of third week which revealed no abnormality in the end to end anastomosis suture line, a repeat fibroptic laryngoscopy at the same time, however, once again revealed the cords in paramedian position: bilateral recurrent laryngeal nerve palsy. Subsequently, the patient was discharged after 3 weeks with tracheostomy tube in situ (). The patient is in regular monthly followup with ENT department with no untoward incident to report. |
The patient in this case report is a female in her early 30s who presented to the emergency department at McLaren Macomb medical center for evaluation of chest pain. The pain started suddenly an hour and a half prior to her arrival to the hospital while she was sitting on the couch. She admitted to two episodes of vomiting as well as bilateral upper extremity paresthesias but denied any other symptoms. Her paresthesias symptoms were numbness and tingling that occurred on and off since her onset of chest pain. She has a recent history of childbirth by vaginal delivery approximately two weeks prior to presentation. Her pregnancy and delivery were uneventful and she had been doing well prior to the symptoms that started on this day.\nThe patient had a past medical history that consisted of anemia and hypothyroidism. She had a previous surgery history of dilation and curettage but denied other surgeries. Her only home medication was a thyroid medication. She denied any history of tobacco, alcohol, illicit drug use or any history of risk factors for cardiac disease. Physical examination of the patient did not show any significant findings. Her vitals were within normal limits and she was in no acute distress.\nDiagnostic testing in the emergency department showed abnormal findings of elevated troponin level of 12.5 ng/ml, which at the facility considered normal levels to be less than 0.039 ng/ml. Troponins are enzymes that are measured in the blood stream and when are present, it suggests that there is heart damage. CT pulmonary angiogram was obtained and the results were unremarkable for pulmonary embolism, a condition where there is blood clots in the lungs. Initial EKG obtained on presentation showed ST depression in the inferior leads with possible prior anterior-septal infarction. (Figure 1) The inferior leads are II, III and AVF on an EKG as delineated by the blue arrows on the figure. Although this does not mean there is a heart attack occurring at this time, it does mean that there is ischemia to the heart muscle.\nThe patient was taken to the catheterization lab by the cardiology team. The result of the procedure was consistent with spontaneous dissection of the right coronary artery. (Figure 2) There was no intervention performed. The cardiothoracic surgeon who decided that there is no need for surgical intervention also evaluated the patient. She was medically managed and discharged home with aspirin, atorvastatin, metoprolol, and Plavix. Her hospital course was uncomplicated and she was discharged home in stable condition. |
An 85-year-old Caucasian male was admitted after sudden onset of expressive aphasia and weakness in both legs lasting 20 seconds. He was athletic, self-reliant and had no cognitive impairment. During the last 28 years, he had experienced 8-10 heterogeneous episodes of acute neurological symptoms, such as central facial palsy, hemiparesis, and non-fluent aphasia, lasting from seconds to 3-4 hours. Precerebral duplex and electrocardiography (ECG) were performed several times with normal results, and EEG registration and 24-hour Holter monitoring had been normal. Previous MRI scans showed no abnormal restricted diffusion, as seen in acute cerebral infarcts, but infarct sequelae in the left temporal lobe and both thalami. Several years later, three additional infarct sequelae were detected in the cerebellum. The patient was treated with platelet inhibitors, and medications and dosages were adjusted after new episodes. There was no suspicion of lack of compliance. Except from age, migraine, and previous smoking, with cessation 35 years ago, he had no known risk factors for cerebrovascular disease. On the current admission, he presented with reduced motor speed in his left arm and leg. Electrocardiography and Holter monitoring showed no signs of atrial fibrillation. CT and MRI revealed multiple, cortical infarct sequelae in the anterior and posterior circulation territories of both hemispheres, and MRI also detected two acute embolic infarcts in the right occipital lobe and one in the left parietal lobe (Figure ). CT and MRI angiograms and duplex sonography did not show significant plaques or stenoses, and pre- and intracerebral flow were normal with asymmetrical vertebral arteries, which were considered a normal anatomical variant. Cortical infarcts in several vascular territories strongly suggest cardioembolic etiology, but transthoracic echocardiogram showed no cardiac sources of emboli, and there was no sign of left atrial enlargement, which may be seen in the presence of atrial fibrillation. The patient concurred to further diagnostic tests aiming to determine the cause of recurrent cerebral emboli, although he was informed that the results would not necessarily alter treatment recommendations. We performed a transcranial Doppler (TCD) bubble test with 10 mL air-mixed saline injected into the left cubital vein while the left middle cerebral artery was insonated with a 2-MHz probe. Injection at resting state produced no microembolic signals, while injection after Valsalva maneuver resulted in a shower of microembolic signals followed by single signals persisting for over 30 seconds. The result implied the presence of a latent right-to-left shunt, and transesophageal echocardiography verified a large patent foramen ovale (PFO; Figure ). In agreement with the patient, we decided on non-operative treatment. Due to previous failure of antiplatelet treatment, we changed to a direct oral anticoagulant (dabigatran 110 mg twice daily), intended as a lifelong treatment. He had no subjective complaints at discharge. |
A 75-year-old male patient visited the hospital complaining of dysphagia and weight loss (as much as 5 kg) that had occurred 3 months prior to this visit. Twenty years earlier, he had a received craniotomy on the left frontal parietal region because of traumatic subdural hemorrhage from falling and had recovered without sequelae. He had taken drug treatment for hypertension, but has no difficulty in daily living activity. For his dysphagia, he complained of more difficulties in swallowing solid foods rather than liquid foods and this was gradually progressing. Seven months after the onset of dysphagia, he was hospitalized, at which time he could only take small amounts of liquid foods instead of regular meals and his weight loss during 10 months since the onset of dysphagia was approximately 10 kg. The patient did not complain of speech disturbance or dyspnea and both movements of the tongue and gag reflex were normal. On his brain MRI scan, partial encephalomalacia was observed on the lesion where the left frontal parietal craniotomy had performed and left inferior temporal gyrus; this was not different compared with a prior brain MRI scan, and any additional lesions which might induce dysphagia was not observed. An endoscopic examination was performed with suspicion of a pharyngolaryngeal tumor, but no abnormal findings were detected. There were also no abnormal results in routine blood and tumor marker tests. As excessive osteophytosis on the anterior part of the cervical vertebrae from C3rd to C6th levels were observed in the cervical plain radiograph, a 3D cervical CT was performed and the identified posterior part of the pharyngolaryngeal cavity was compressed and narrowed by osteophytes from C2nd to C7th vertebrae levels (). As the dysphagia by anterior cervical osteophytes was suspected, a VFSS (Video Fluroscopic Swallowing Study) was performed (). Findings revealed that a movement of the epiglottis displayed an abnormal recurvation by osteophytes, with at least 50% of food remaining in the epiglottic valleculae. Pyriform sinuses were observed in all tested materials, and asymptomatic aspiration was shown as 8 points of the PAS (Penetration-Aspiration Scale) in 2 cc of diluted barium swallowing test. After the above examinations, an electronic stimulator, VitalStim® (Chaltanooga group, Austin, USA), was conducted one hour a day for 10 days. The treatment sessions were completed in two weeks. The electronic stimulation used a 2-channel form with an alternating current, wherein one of the electrical pads of the first channel was attached between the musculus biventer mandibulae muscle and hyoid bone and another electrical pad was attached between the hyoid bone and thyroid cartilage. The electrical pad of the second channel was attached between the thyroid and cricoid cartilages and vertically to the inferior part of the cricoid cartilage. A week after completing the electronic stimulation treatment, a VFSS was performed, where it was found that aspiration and nasal regurgitation had disappeared and the remaining amount of food shown in the epiglottic valleculae and pyriform sinuses were reduced to below 10%. Thus, it was possible to change his diet from liquid form to regular meals. After one month, however, dysphagia began to progress again and aspiration pneumonia had developed, which required hospitalization. Compared with previous findings, VFSS results revealed an increase in the amount of retention of contrast in the epiglottic valleculae and pyriform sinuses by 10-50% and the movement of epiglottis had decreased in all tested materials. It was suggested that his symptoms has worsened compared to previous findings. Due to persistent dysphagia, his diet was changed back to liquid food. He also received consultation in our department of neurosurgery for operation due to severe general weakness caused by insufficient nutrition. Next, he decided to have an operation after 2 year 7 months since the onset of dysphagia. Osteophytes cervical vertebrae from C2nd to C7th levels were removed via an anterior approach (). After the operation, he recovered without any side effects and 1 month later, VFSS was performed. Based on the nasal reflux that had been observed in the swallowing water test disappeared and food remaining in the epiglottic valleculae and pyriform sinuses were below 10%. In spite of the formation of the inner wall membrane, the remaining food was removed by chin tuck and repetitive swallowing. It was determined that the epiglottic motion had improved and the remainder in the epiglottic valleculae and pyriform sinus had reduced remarkably (). The patient was able to return to a general diet, had gained approximately 6kg of weight, and was satisfied with his postoperative improvement of symptoms in spite of having subjective dysphagia for waterless biscuits. |
An 87-year-old Caucasian man presented to our institution with chest and epigastric pain radiating to his back. Computed tomographic scans were performed urgently, and these showed a large Crawford type I thoracoabdominal aortic aneurysm (Figure ). The aneurysm measured 17 cm in length and was 5 cm to 6 cm distal from the left subclavian artery and 2 cm to 3 cm proximal to the celiac axis. The maximum anteroposterior diameter of the aneurysm was 13 cm above the diaphragm. The aorta between the celiac axis and the renal arteries was of normal size. Another aneurysm measuring 6 cm in length and 4 cm in diameter with no extension to the iliac arteries was detected distal to the renal arteries.\nOur patient was not in a fit condition to undergo open surgery, so endovascular surgery was the preferred option. The diameters of the proximal and distal necks of the aneurysm were 3.2 cm and 3.4 cm, and a large mural thrombosis was present along all its length. We decided to treat our patient with a single tube stent graft. Although a long segment of the aorta would be covered during the procedure, we considered that there was a low chance of spinal ischemia, as the mural thrombosis had plugged all of his intercostal and lumbar arteries. Cerebrospinal fluid drainage was considered as a protective move for spinal circulation during the procedure.\nUnder general anesthesia, our patient's right femoral artery was dissected and controlled. An endovascular stent graft (VALIANT TF 4242C200X, Medtronic) was deployed distal to the left subclavian artery, thus covering the aneurysm. The stent graft was 21 cm in length and was placed just above his celiac axis. We controlled the proximal end deployment by real-time transesophageal echocardiography, and the distal end deployment under angiography. Follow-up transesophageal echocardiography, computed tomography and angiography showed a complete exclusion of the thoracoabdominal aneurysm (Figure ). Correction of the abdominal aortic aneurysm was programmed for later. The patient was discharged three days after the procedure and showed no complications during the succeeding nine months. |
A 57-year-old female visited a respiratory internal physician due to suspected lung cancer (based on a mass screening chest X-ray examination). She did not have any symptoms. The chest X-ray showed a tumor shadow in the upper-middle field of the right lung with pleural effusion and a tumor shadow in the upper field of the left lung (). Computed tomography (CT) of the neck and chest revealed that the tumor shadows had been caused by a substernal goiter connected to the thyroid gland in the neck. According to the patient, she had been diagnosed with a goiter about 23 years ago, and it was followed up, but the follow-up process had been discontinued several times. After about 20 years, she visited our hospital for surgical treatment.\nIn a physical examination, the palpable thyroid gland was found to be diffusely swollen and soft and exhibited poor mobility. The lower pole of the thyroid was not palpable.\nA blood examination revealed normal thyroid function, a thyroglobulin level of 352 ng/ml, and negativity for the thyroglobulin antibody.\nUltrasound showed that the cervical thyroid gland was diffusely enlarged and exhibited multiple regions of cystic degeneration, but no obvious malignant findings were observed.\nCT of the neck and chest () showed the diffusely swollen thyroid gland and a substernal goiter, which extended to both sides of the thorax. Specifically, it extended to the bifurcation of the trachea on the dorsal side of the superior vena cava, the innominate vein, the aortic arch, and the ventral side of the trachea. The width of the goiter at the mediastinum was 145 mm (length: 80 mm, thickness: 80 mm). The right side of the substernal goiter was bigger than its left side. The interior of the lesion was heterogeneous, and calcification was seen in part of it. The goiter had compressed the trachea in the mediastinum, and the lumen of the trachea measured 6 mm in diameter at its narrowest point. Pleural effusion was noted in the right thorax. We performed 18F-fluorodeoxy glucose positron emission tomography to determine the malignancy of the substernal goiter, but no radiotracer accumulation was observed.\nWe also conducted a pathological examination. Fine-needle aspiration cytology of the cervical thyroid gland resulted in the lesion being classified as of “indeterminate significance,” and a pathological examination of a needle biopsy sample from the same site led to the lesion being diagnosed as a follicular neoplasm. Fine-needle aspiration cytology of the right pleural effusion demonstrated that it was benign.\nThe patient underwent total thyroidectomy using a transcervical and full sternotomy approach. The anesthesiologist intubated the patient with a bronchoscope. Although tracheal stenosis was observed, intubation was performed smoothly. Later, the tracheal tube was replaced with an NIM™ EMG endotracheal tube so that intraoperative nerve monitoring could be performed. The patient was placed in a supine position with her neck well extended. A cervical skin incision was made, and a median chest midline incision and full sternotomy were performed. First, we identified the bilateral vagal nerves and confirmed the absence of paralysis with the NIM™. As a preparation for the resection of the substernal goiter, the major blood vessels, including the innominate vein, brachiocephalic trunk, superior vena cava, and left subclavian artery, were carefully separated from the substernal goiter, and then thyroidectomy was performed ().\nThe right superior thyroid pedicle and right middle thyroid vein were ligated and dissected to allow the right thyroid lobe to be rotated to gain a view of the recurrent laryngeal nerve (RLN) from the lateral aspect of the thyroid gland, but the goiter prevented the right thyroid lobe from being rotated. It was difficult to identify the right RLN, so we decided to try to exteriorize the left thyroid lobe, which was smaller than the right thyroid lobe. The left superior thyroid pedicle and the left middle thyroid vein were ligated and dissected. The left thyroid lobe was more mobile than the right thyroid lobe, and the left RLN could be identified by rotating the left thyroid lobe in the medial direction. The NIM™ was effective at identifying the RLN. After identifying the left RLN, the left lower thyroid artery was ligated and dissected. The left RLN was carefully separated from the dorsal side of the left thyroid lobe and the substernal goiter so as not to cause any damage. The substernal goiter, which was connected to the left thyroid lobe, was pulled in the cranial direction, and the part adhering to the surrounding tissue, particularly the tissue between the goiter and the innominate vein, was dissected by ligation and coagulation with an energy device. Subsequently, the left thyroid lobe was also separated from the trachea. The exteriorization of the left thyroid lobe improved the mobility of the right thyroid lobe, and the right RLN was identified by dislocating the right upper pole to the caudal side. We carefully separated the right RLN from the goiter and ligated and dissected the right lower thyroid artery. We pulled the substernal portion of the right thyroid lobe gradually; separated the tissue connected to the goiter, including the left thyroid lobe; and succeeded in moving the substernal goiter in the cranial direction. The remaining attachments between the right thyroid lobe and trachea were broken, and a total thyroidectomy was conducted. We found three parathyroid glands had adhered to the resected thyroid gland, so we performed autotransplantation using the sternocleidomastoid muscle. The wound closed after drains were inserted in the neck and mediastinum. After the surgery, the patient was extubated immediately because no respiratory tract problems (e.g., tracheomalacia) were noted. The total duration of the operation was 9 h and 22 min, and the total amount of intraoperative blood loss was 3298 ml. The resected thyroid weighed 614 g ().\nPostoperative transient hypoparathyroidism was observed. Routine treatment with calcium (3 g daily orally) and 1 alpha-hydroxyvitamin D3 (2 μg daily orally) was administered. The patient was discharged home on the 9th postoperative day on levothyroxine (100 μg daily orally). A histopathological examination did not reveal any signs of malignancy, and so the lesion was diagnosed as an adenomatous goiter. |
A healthy 40-year-old woman visited our clinic with complaints of ocular discomfort in both eyes. There was no documented history suggestive of connective tissue disease or any systemic disease. Six months prior to this presentation, she had undergone a bilateral regional conjunctivectomy to treat a chronic hyperemic conjunctiva. Following this procedure, she was started on a regimen of 0.2 mg/mL (0.02%) MMC eye drops four times daily for 5 days. The patient's best-corrected visual acuity was 20 / 25 in both eyes. Based on the results from the slit-lamp examination, the conjunctiva and Tenon's capsule on the nasal and lateral bulbar area were excised. Slit-lamp examination also revealed marked scleral thinning and a bluish hue due to seeing the underlying choroid layer which measured 8 × 4 mm in the temporal portion of her right eye and 8 × 3 mm in the temporal portion of her left eye (). There were a 3 × 1.7 mm sized yellowish-white calcified plaque on the nasal bulbar conjunctiva of her right eye and a 1.5 × 0.5 mm sized yellowish-white calcified plaque on the nasal bulbar conjunctiva of her left eye (). In addition, we found that the bare sclera was covered with a thin abnormal membranous tissue. There were no fluorescence stained lesions overlying the membranous tissue in either of her eyes (). She began conservative treatment with antibiotics and artificial eye drops. The condition of the calcified plaque seemed to stabilize over the course of the next several weeks, and the area of scleral melting did not show any further deterioration. However, there was a newly developed fluorescence stained lesion on the calcified plaques (). Fifteen months later, the thinned scleral area and fluorescence stained lesions showed no significant interval change (). Because the scleral melting did not show any further deterioration during the following fifteen months, and aggressive surgical intervention might not have been helpful for improving her lesions, we decided to continue regular follow-ups and non-surgical treatment. |
An 81-year old female with a history of coronary artery disease, hypertension, and thrombocytosis suffered a witnessed trip and fall onto a nightstand. The patient took 75 mg of clopidogrel daily in addition to an 81 mg aspirin tablet. She reported a mild headache however had no change from her baseline mentation per family members with no evidence of obvious injury aside from a small area of ecchymosis near a small forehead laceration. She remained up and ambulatory with no further complaints. Ten hours after her injury the patient presented to the Emergency Department with stridorous and agonal respirations with a profoundly decreased level of consciousness. She was noted to have developed extensive ecchymosis on the anterior portion of her neck and chest. Her symptoms had begun rapidly shortly prior to arrival while lying in bed. Family reported that she had been in the constant company of her husband with no further falls or injuries that had occurred since her fall. The patient was intubated upon hospital arrival due to respiratory extremis with obvious swelling and crepitus noted on neck examination. A noncontrast CT scan of head was unremarkable while there was demonstration of a large retropharyngeal hematoma measuring 3.6 cm by 5.3 cm by 20 cm on a CT of the cervical spine with no evidence of fracture. Her hemoglobin was 9.5 gm/dL and platelets were 1234 per deciliter, with an INR of 3 and a slightly below normal and activated partial thromboplastin time of 23.9 seconds (reference range 25-35 seconds). A CT angiogram of the neck was subsequently obtained demonstrating active bleeding from the anterior ligaments of the vertebral column that was not felt to be amenable to embolization (). Given the extent of the hematoma intraoral surgical evacuation was performed with bleeding from the anterior vertebral spine controlled with Bovie cauterization, placement of topical thrombin, and drain placement. No reaccumulation of hematoma was noted during her hospital course. The patient unfortunately expired 12 days from the date of admission from presumed aspiration pneumonia and multisystem organ failure. |
A 4-month-old baby girl was admitted into our hospital with a mass on the left side of her mouth that had been detected for over a month. One month before her presentation at the hospital, the infants parents noticed a mass approximately 5 cm × 4 cm × 4 cm in size on the left side of her mouth. A local hospital provided a diagnosis of mumps, which turned out to be incorrect. The mass continued to gradually increase in size over time. Therefore, the parents came to our hospital for additional diagnosis and treatment. A slight swelling was visible on the left side of the infants face, and a mass of 5 cm × 4 cm × 4 cm in size filled the left side of the oral cavity. It was difficult for the infant to close her mouth (Fig. ).\nBased on the patients history, the infant was normal at birth and no pregnancy or delivery complications were noted. On physical examination, the infant appeared well-fed and had no apparent history of medication or surgery, nor did she have any known allergies. After admission (2018.12.28), a head MRI scan was performed. The MRI indicated the presence of a large mass with abnormal signal in the left oral and maxillofacial region, which expanded into the peripheral area. The T1WI image revealed a low signal, the T2WI showed a high signal, and the diffusion of DWI was limited. It was observed that the mass was noticeably different from the peripheral tissue on enhanced MRI, and the size of the mass was 54 mm × 39 mm × 53 mm. The left maxillary, alveolar bone, and maxillary sinus wall were partly involved (Fig. ). Histopathology revealed the presence of clusters of small round cells in the fibrous tissue that were melanin pigmented. Immunohistochemical staining demonstrated that the cells were positive for cytokeratin (CK) EMA, HMB-45, neuron-specific enolase (NSE), SYN, and VIM (Fig. ). The cells were negative for glial fibrillary acidic protein (GFAP), S100, CD99, DES, LCA, SOX10, SMA, TDT, and CD34 (Fig. ). Combined morphology and immunohistochemistry confirmed that the tumor was a “melanotic neuroectodermal tumor of infancy”. Therefore, complete resection of the tumor was performed under general anesthesia, with a 2 mm margin around the tumor (Fig. ). Unfortunately, 3 months later (2019.4.9), the patient was readmitted to our hospital, and the MRI of the nasopharynx demonstrated the presence of a new mass of abnormal signal in the same region as before. The size of the mass was 28.8 mm × 28.3 mm × 11.6 mm. It was clear that the tumor had recurred (Fig. ).\nConsidering the young age of the patient and excessive trauma of a second surgery, we suggested metformin (Sino-American Shanghai Squibb Pharmaceuticals Ltd, 0.5 g/ tablet) as a treatment for the tumor. We prescribed an oral dose of 7 mg/kg to be given 3 times a day after meals.\nAt the 9-month follow-up examination, the parents reported no remarkable discomfort in the infant due to the medication. The MRI of the nasopharynx region (2020.1.8) revealed that the size of the tumor was unchanged (Fig. ). Our observations indicated that the tumor was well controlled, and the growth and appetite of the infant were normal (Fig. ). The parents were recommended to continue oral metformin treatment for the patient and to have regular follow-up examinations. |
We are reporting about the case of a 22-year old woman, who made a first visit as an outpatient with pain that arose in acute form in the area of the left groin. The complaints occurred in acute form without preceding trauma and led to inability to put stress on the left leg. Until the occurrence of this acute pain the patient was free of complaints and active in sports.\nThe physical examination produced pain on pressure in the groin as well as pronounced pain on movement of the left hip joint mainly during inner rotation. There were no neurological deficits. Soft tissue, blood circulation and sensorimotor function of the lower extremities were intact. Similarly there were no signs of an acute infection or general disease symptoms such as fever, night sweat or loss of weight. The ultrasound of the inguinal region carried out subsequently revealed an intraarticular exudate of the left hip joint, so that conventional X-ray examination was initiated.\nIn the overview of the pelvis and of the axial image of the left hip joint an osteolytic lesion appeared in the area of the proximal femur with partially compartmented portions and reactive peripheral sclerosis. In addition a pathological fracture was remarkable in the area of the calcar, which explained the complaints of the patient. (Fig. )\nAn MRI of the pelvis produced a large, cystic space occupying lesion of the neck of thigh [collum femoris] and proximal femur without sign of malignant degeneration. (Fig. )\nThe blood test showed an increase of the alkaline phosphatase to 115 U/l (reference value: 35–104 U/l) otherwise with parameters of normal value.\nRelying on differential diagnosis an aneurismal bone cyst, giant cell tumor and juvenile bone cyst were discussed.\nOwing to the pathological fracture, finally the indication of operation seemed sensible and it was carried out by bone biopsy, curettage, spongiose plastic surgery and internal fixation (DHS). (Fig. )\nPostoperatively the histological examination of the bone material taken intraoperatively made the diagnosis of fibrous dysplasia, which was not taken into consideration in the preoperative considerations during differential diagnosis.\nFor the assessment of the distribution pattern in conclusion bone scintigraphy was still performed, according to which a monoostotic form of fibrous dysplasia affected the patient. |
A 76-year-old otherwise fit and healthy female presented at the Accident and Emergency Department at 2:15 am with the complaint of a mildly painful and remarkably swollen tongue that developed after eating mint chocolate. Although the patient had tenderness and the frightening swelling in the mouth causing discomfort and moderate difficulty in swallowing, she had no shortness of breath or any other complaint. She gave a medical history of rheumatic polimyalgia in the past requiring no medical treatment at present. She remembered a previous allergic reaction to mint.\nOn examination it was noted that a remarkable swelling was localised only on the lefthand side of the tongue (please see Figure ). The tongue seemed to be suffused and a superficial non-tender ulceration was seen at the anterior quarter of the surface. There was a mild tenderness and a loss of taste on this side. The right-hand side of the tongue was completely normal. Tongue movement was normal apart from mild restriction caused by the swelling. No other pathology on the oral mucosa or in the throat was observed.\nThere was no skin rash or any other systemic reaction, and the vital parameters were stable. Blood results showed a mild elevation of C-reactive protein (CRP = 17) and erythrocyte sedimentation rate (ESR = 33).\nAs the symptoms were considered as a local allergic reaction, the patient was given 4 mg chlorphenamine orally and 100 mg hydrocortisone injection intramuscularly. The swelling responded to the medication quickly and the patient was discharged after a period of observation.\nWe made a follow up after one and three months. However, although the swelling had almost completely gone by the next morning and the tongue had become pain free, the follow up revealed that the healing process was indeed longer. Even after three months a scar was seen on the top of the left-hand side of the tongue (please see Figure ). No other disability was reported and the sense of taste had also returned to normal. |
A 64-year-old woman underwent liquid silicone injections for augmentation mammoplasty 42 years previously. Eight years prior to admission, siliconomas were removed due to discomfort. She visited a hospital with the chief complaint of a painful mass in her left breast. The mass was resected and a histopathological examination revealed the tumor to be an invasive micropapillary carcinoma. The surgical margin was positive for malignant cells and she visited the hospital for further treatment. She was a healthy-looking woman. The left breast was craggy and it came in contact with the axilla, which thus made it difficult to palpate the tumors. No breast tumor was palpable on the other side. The laboratory parameters did not show any abnormalities and there was no evidence of distant metastasis. She was not on any medication. She had never taken oral contraceptives nor received hormonal therapy. She had experienced three pregnancies and delivered once. Her family history revealed no malignancies.\nA subsequent dynamic magnetic resonance imaging (MRI) examination with Gadolinium (Gd)-DTPA enhancement demonstrated the four tumor shadows with similar enhancement at distant portions. Because it was unlikely that four malignant tumors existed at the same instant, they were thus considered to be coexistent malignant tumors and siliconomas. Ultrasonography revealed masses with an irregular shape and contour, extensive hypoechogenicity or shadowing. The tumors with a heterogeneous internal echo with a slight degree of Doppler signaling were considered to be malignant tumors; those with homogeneous internal hypoechogenicity with no Doppler signaling were considered to be siliconomas.\nA left-sided mastectomy and complete axillary lymph node dissection was thus performed. The histopathological findings of the mastectomy specimen were as follows. The siliconomas were observed to be spread around the operational scar. Three tumors were identified, all in immediate contact with the siliconomas as indicated by ultrasonography (Figure ), which measured 12 mm on the upper side of the breast, 3 mm on the lateral side and 20 mm on the subareolar area. A tumor measuring 9 mm in diameter was located on the medial side, but had no connection with the siliconomas (Figure ). In each tumor, neoplastic cell clusters floating within clear spaces defined by a network of loose fibrocollagenous stroma were recognized (Figure ), and the tumors were diagnosed as IMPCs. Scirrhous carcinoma components were also seen in each tumor. The malignant cells of the three tumors had contact with collections of rounded vacuoles of varying sizes (Figure ). Lipid droplets were contained in these vacuoles along with macrophages and foreign-body giant cells. In addition, lymphatic invasion was observed in all tumors and perineural invasion was seen for the medial tumor. The tumor in the subareolar area reached the fat tissues outside of the gland, the dermis and the larger muscle. Eleven of sixteen axillary lymph nodes showed tumor involvement. The histological grade, based on a modified Bloom Richardson scoring system, was intermediate. The scores for each parameter (tumor tubule formation, number of mitoses and nuclear pleomorphism) were 3, 1 and 2, respectively. Immunohistochemically, the tumors were estrogen receptor (ER) and progesterone receptor (PgR) positive and C-erbB-2 negative. Postoperatively, since the patient consistently refused to be treated with adjuvant systemic chemotherapy, radiotherapy was administered with 50Gy to the chest wall. Subsequently, endocrine therapy was administered using antiestrogens. Three years after the operation, no metastasis was recognized in any organ. |
A ten-year-old girl was admitted to our general hospital with numbness of her left palm and fingers in the last 5 months before admission. At that time, she was hit by a car while she was riding a bicycle. The car was coming from opposite side, and she fell with her left forearm was sliced by licensed plate of the car. There was a semicircular open wound with active bleeding on the left forearm, and she was in pain. She was brought to a nearby clinic and had her left forearm sutured. After the pain subsided, she felt numbness of her left hand and fingers. In addition, she could not extend her fingers. Finally, the patient decided to seek medical attention and get further treatment at our general hospital.\nFrom physical examination, there were claw hand deformity with thenar and hypothenar atrophy as well as a scar on the anterior side of distal forearm (). Sensorium loss of the palm and third, fourth, and fifth fingers was impaired. No tenderness was found. Capillary refill of the fingers was normal. Range of motion of the fingers was altered with limitation of finger abduction and thumb apposition (). Moreover, range of motion of the wrist was within normal limit.\nRoutine laboratory examination was within normal limit. The patient was taken for wrist and forearm radiographs and, similarly, there was no abnormality depicted on either bones or soft tissue.\nThe patient also underwent electromyography examination which showed median and ulnar nerve lesion at the left forearm with total axonal degeneration. No signs of reinnervation of both peripheral nerves were detected.\nThe patient was diagnosed as ulnar and median nerve palsy of left forearm, and then we planned to perform surgical exploration of the nerves and to repair with sural nerve graft, Zancolli procedure and sural nerve graft.\nIntraoperatively, skin incision was made on the previous surgical scar. Injury site was explored, and complete rupture of both ulnar and median nerves was found. Degeneration of both nerves was also seen, with neuroma rising from both the proximal stumps. The proximal and distal ends of both ulnar and median nerves was cut until nerve fascicle was visible. The distance between proximal and distal stump was measured: for ulnar nerve the distance was 7 cm, while it was 8 cm for median nerve. Sixteen centimeters of ipsilateral sural nerve was harvested, and the ulnar and median nerves were repaired using the nerve graft. Then Zancolli procedure was performed: skin incision was made along the palmar crease, A1 pulley was identified around metacarpophalangeal joint, longitudinal incision was made on the pulley, flexor digitorum superficial tendon was retracted laterally, metacarpophalangeal joint capsule was identified, an elliptical incision was made over the joint capsule, and capsulodesis was performed. Postoperatively the wound was closed and immobilized by elastic bandage ().\nWe followed the patient at 3-week postoperatively, and the patient had improvement of her claw hand (). She was advised to continue her rehabilitation of her hand to further improve her hand function, especially opposition and key pinch. At 6-month follow-up, she had improved grip strength and normal functional level of her left hand. At 2-year follow-up, she could handle daily activity as before the accident and was satisfactory with her condition. () |
The 77 year old, male patient diagnosed with esophageal cancer underwent esophagectomy and gastric conduit reconstruction in an external hospital in January 2020. The patient suffered from an insufficiency of the intrathoracic anastomosis of the gastric conduit, which was treated by implanting esophageal stents.\nAfter an episode of upper gastrointestinal (GI) bleeding that led to a hemorrhagic shock, the patient was transferred to the tertiary care hospital. Over the next few days, the patient presented further episodes of upper GI bleeding, which could not be controlled by endoscopy. In a CT scan we identified a pseudoaneurysm of an intercostal artery dorsal of the gastric conduit (see Fig. ). After an interdisciplinary discussion, we decided in favor of an endovascular therapy for the pseudoaneurysm.\nThe angiography was performed under general anesthesia. Access was gained via a retrograde 6 French sheath in the right common femoral artery. The intercostal artery of the right, eighth intercostal space was probed with a 6 French Guider Softip XF Catheter (Boston Scientific). Digital subtraction angiographies (DSA) were made from different projections. The maximum diameter of the pseudoaneurysm was 8 mm (see Fig. ).\nThe DSA images also showed a fine, vertical vessel projected onto the spine, highly suspicious of being the anterior spinal artery. The direct connection between that vessel and the intercostal artery could not be identified clearly. Also we were not able to evaluate with certainty whether this vessel would be collateralized from the opposite side if we embolized the intercostal artery completely.\nConsequently, we decided to implant a stentgraft into the intercostal artery that covered the pseudoaneurysm but maintained the blood flow within the artery and all uncovered side branches of the artery. Because of the small diameter of the intercostal artery (3 mm), we chose the PK Papyrus Coronary Stent (Biotronik, 3 mm x 15 mm).\nFirst, we had to find a stable position in the ostium of the intercostal artery using the 6 French Guider Softip XF Catheter. Then, the intercostal artery was probed with a 0.014” guide wire. Finally, the balloon expandable stent was placed safely and precisely in the intercostal artery to cover the pseudoaneurysm. In the final DSA, the pseudoaneurysm could no longer be delineated while the perfusion of the anterior spinal artery was preserved (see Fig. ).\nThe postinterventional clinical course in the tertiary care hospital was uneventful without further episodes of upper GI bleeding. Unfortunately, during the preparation of this case report, we received the news that the patient had passed away in another hospital a few weeks after the intervention. |
A 60-year-old male was referred to our department, complaining about gradually worsening hoarseness, during the last 8 month period. Occasional dysphagia and foreign-body sensation were also reported upon referral. The patient was a heavy smoker for more than 20 years, reporting an average of 20 cigarettes per day. Alcohol was also a factor, and although no real alcohol abuse or indulgence was noted, the patient was a rather frequent user.\nMedical history only revealed arterial hypertension under treatment with beta blockers. Haematological and biochemical tests did not show any significant abnormalities.\nPhysical examination included a full head and neck examination, complemented with flexible fiberoptic laryngoscopy. Typical ear, nose and throat examination did not reveal any abnormal findings and neck palpation was negative. However, fiberoptic laryngoscopy revealed a lesion affecting both vocal cords and anterior commissure, while vocal cord mobility appeared impaired. On these grounds, a cervicothoracic and upper abdomen computed tomography (CT) scan with intravenous gadolinium was decided and the patient was scheduled for direct microlaryngoscopy and biopsy of the lesion under general anaesthesia.\nImaging confirmed the laryngeal lesion, yet it also indicated a second lesion about 2 cm below the inferior end of the primary one, arising somewhere between the first and second tracheal ring. Intermediate tissue appeared grossly normal (). No signs of enlarged cervical lymph nodes were noted and laryngeal cartilages showed no abnormal findings.\nOn the other hand, histopathological examination after biopsy of the lesion under general anaesthesia confirmed the diagnosis of squamous cell carcinoma. The lesion was carefully mapped and proved to be a glottic carcinoma affecting the anterior commissure and appearing in strong correlation with the thyroid cartilage. The lesion infiltrated the left and the first tertile of the right vocal cord. No subglottic extension was noted. In this context, the patient was informed and consent for radical surgical therapy was obtained.\nThe patient underwent total laryngectomy and wide excision of the trachea which included the second tumour within safe limits (). The procedure was complimented with left thyroid lobectomy and bilateral selective neck dissection (Robin’s levels II–IV). Paratracheal lymph nodes (Robin’s level VI) were also carefully dissected. The overall postoperative course was uneventful. The patient was discharged from our department on day 16 with very good swallow function and was decannulated after 1 week. Surgical resection was followed by postoperative radiation therapy (6400 cGy/32 fraction).\nThe final pathological report was of crucial importance in our case. First of all, the surgical margins of resection were found to be free of disease. Second, histological sections from the tumour of the glottis showed the characteristic morphology of squamous cell carcinoma. Cancer cells were large in size and polygonal in shape with eosinophilic cytoplasm and nuclei with moderate variation in size and shape. There were a moderate number of mitoses and keratinisation could be focally observed. Cancer cells showed an infiltrative pattern consisting mainly of nests and trabeculae that invaded the vocalis muscle in both the vocal cords. The perichondrium of thyroid cartilage was focally invaded by cancer cells. Histological sections from the tumour of the trachea showed morphological features identical to those of the tumour of the glottis. An upward infiltrating pattern could be noticed. Moreover, a comparative immunohistochemical study of the two tumours showed strong positivity of cancer cells in stains for keratins AE1/AE3 and 34βΕ12 and moderate positivity in stains for CK5/6, CK8/18 and epithelial membrane antigen. Immunohistochemistry for D2-40 antigen (podoplanin) illustrated the positivity of the lymphatic endothelium. Immunohistochemical stains for other vascular endothelia (CD31 and CD34 antigens) were also performed, and were negative. In the region between the two tumours, many lymphatics containing neoplastic emboli could be observed (). Finally, two tumour-infiltrated lymph nodes (the larger being of 1.2 cm diameter) with extracapsular spread were found in the left neck dissection specimen. A pT4a(m)N2b stage, according to eighth edition TNM staging, was established. |
A 65 year old man presented with a growth in the upper left alveolus with nasal obstruction for the past 6 months. He also complained of a mild intermittent pain associated with sudden ulcerated growth on the left maxillary alveolar ridge. He had undergone extraction of upper left posterior tooth 5 years back. He did not give any contributory medical and family history. He did not chew or smoke tobacco or use betel nut. On extraoral examination a non-tender, firm swelling involving the left side of face extending from infraorbital rim till 2 cms short of lower border of mandible superoinferiorly []. Intraorally the surface of the growth was covered with a yellowish necrotic material and it was slightly tender on palpation. It was extending to the buccal vestibule and the palatal side of alveolus in the region extending from tooth number 25 till left maxillary tuberosity region []. There were no palpable lymph nodes. The clinical differential diagnosis included the most common malignancies in the oral cavity such as squamous cell carcinoma (SCC), minor salivary gland tumor, and carcinoma of the maxillary sinus. Investigations revealed normal complete hemogram, blood sugar, and liver and kidney function tests. The patient's Enzyme- linked immunosorbent assay for Human immunodeficiency virus and Venereal Disease Research Laboratory were negative. Scrapings taken from the involved region to rule out fungal granulomas did not show any fungi. On radiographic examination in a Waters and Caldwell view, opacification was seen in left maxillary sinus with destruction of anterior and posterolateral wall of maxillary sinus. Computed tomography (CT) scan was performed and sections were taken in the axial, coronal, and sagittal planes. Axial section revealed a large soft tissue density lesion with its epicentre in the maxillary sinus, causing destruction of all walls of the maxillary sinus, extending medially into the lateral wall of the nasal septum, left nasal cavity involving the inferior and middle turbinates, laterally into the pterygopalatine fossa and inferiorly causing destruction of the hard palate and alveolar processes of the left maxilla in the molar region []. Next a biopsy was taken for histopathological evaluation, which was suggestive of peripheral reparative giant cell granuloma. In doubt, a repeat biopsy for immunohistochemistry findings was done. Immunohistochemistry reports were suggestive of non-Hodgkin's lymphoma diffuse large B cell type[] with tumor cells positive for CD20 []. The patient was staged IE according to the Ann Arbor Staging system. He received 6 cycles of standard (R-CHOP) regimen, Rituximab 375 mg/m2 d1 (Day 1),[] Cyclophosphamide 750 mg/m2 d1, Doxorubicine 50 mg/m2 d1, Vincristine 1.4 mg/m2 d1, and Prednisolone 100 mg (Day 1-5). The response to the treatment was successful []. Post treatment the patient is in follow up over past 1 year and no recurrence is seen till date []. |
A 29-year-old Caucasian man was seen in our department for a second opinion because of asymmetrical walking pattern and progressive sourness in his amputated leg. He was referred from a rehabilitation center where a standard roentgenogram showed evidence of a gauze in situ near the distal femur stump with progressive ossifications (Figure ).\nHis medical history noted a severe high energy trauma seven years ago. He had sustained multiple injuries among which a severe crush injury of the left leg with heavy bleeding for which an acute amputation was performed.\nAfter six weeks he could be discharged from the hospital and was measured an advanced prosthetic leg for optimal rehabilitation. It took him 3 months to walk without aids. Two years later he fell on his stump in the bathroom. An X-ray was made of the left upper leg stump to rule out any fracture. The radiologist reported no fracture, but a swelling of soft tissue with calcifications at the femoral cutting edge. The report also mentioned that at the time of roentgenography, the stump was covered with a gauze, because a radio-opaque marker was visible on the X-ray. In the conclusion of the X-ray report, no mention was made on the gauze fibroma.\nHis painful stump recovered in a few weeks but five years later, he consulted his rehabilitation physician for asymmetrical walking pattern with soreness of the stump. A standard roentgenogram revealed progressive calcifications compared to the previous study. The radio-opaque marker was recognized as a retained surgical gauze which was left behind during initial surgery. The progressive ossifications were thought to cause his complaints. He was directed to our department for re-evaluation. Clinical examination showed an otherwise healthy man with a prosthetic leg with vacuum fitting. His walking pattern was slightly asymmetrical due to partial weight-bearing of his artificial leg. His left leg stump showed obvious thickening at the distal end. No painful or sharp masses could be palpated and no skin defects were noted, let alone multiple scars from previous trauma and surgery.\nTo exclude malignant tumor growth, magnetic resonance imaging showed a benign granuloma of 12.6 × 10.0 centimeters with remodelling of the ossal part of the femur around the tumor. This was interpreted as a physiological reaction of the leg to encapsulate the foreign body. The suspected surgical sponge was not clearly identified from the surrounding granuloma.\nMost effective treatment options would be to completely remove the calcifications including granuloma with surgical sponge. The patient however had experienced much advantage from the firm swelling of his stump and did consent. His prosthetic leg fitted well in time because of these progressive ossifications around the gauze. Therefore it was decided to only remove the gauze, but not the ossifications nor the granuloma, to remove the trigger for further progression of the ossifications, but to maintain the form of the stump.\nAt surgery, the distal end of the stump was opened through an old scar. A well organized, firm, brownish tumor was opened which appeared to have a wall of around two centimetres. Brownish, serous fluid was drained and sent for culture of micro-organisms. Remnants of a partly disintegrated surgical sponge of 40 × 40 centimeters were removed with curettage of the granuloma (Figure and Figure ). The wound was closed in layers with vacuum drainage. At the fifth postoperative day the patient developed fever and a swollen painful stump for which incision and drainage of an infected seroma with staphylococcus aureus was necessary. The postoperative course was further complicated by infectious complications and shortening of the femoral end with removal of all calcifications could not be avoided (Figure ). Disturbance of wound healing and treatment with vacuum assisted closure devices characterized the clinical course. Five months after initial removal of the gauze, a definitive stump correction was carried out without complications and a rehabilitation program with a new prosthesis could be initiated. |
A 47 years old male presented with chest pain and gradually progressing dyspnoea (MRC Grade III) of three months duration. He was a labourer and a non smoker with no history of exposure to any occupational or inorganic dusts. The chest pain was dull aching and mainly localized in left axillary region with no radiation and not related to the meals. He was also having cough with mucoid expectoration. There was no hemoptysis. Examination revealed averagely built person with BMI of 20, and no clubbing and lymphadenopathy. Respiratory system examination revealed diminished movements and breath sounds on the left hemithorax. Chest radiograph revealed a uniform density homogenous opacity in the lower left hemithorax with obliteration of the left cardiac border. The mediastinum was shifted to the right side []. CT scan of the thorax revealed a large well defined heterogenous density anterior mediastinal mass. There were some areas of necrosis within the mass. The mass was extending up to the pleural surface, but there was no infiltration of chest wall. There was no involvement of the mediastinal lymph nodes [ and ]. Abdominal ultrasonography did not revealed any organomegaly. Routine blood investigations were within normal limits. Serum cortisol levels were within normal levels. Fibreoptic bronchoscopy did not revealed any abnormality. Bronchial washings, bronchial brush biopsy and transbronchial needle aspiration biopsy of the mass were inconclusive. Trans-thoracic tru-cut biopsy of the mass revealed small round tumor cells, but the exact histological diagnosis remained uncertain. As the diagnosis was inconclusive, the patient was taken up for thoracotomy with excision of the tumor. Intra-operatively it was observed that there was a large firm mass in the mediastinum on the left side with invasion of the great vessels. The mass was extending up to the chest wall. Hence, debulking of the tumor was done to the extent possible. Complete excision of the tumor could not be done as it was infiltrating the great vessels and the pericardium. On gross examination, the tumor was uncapsulated, firm, pink-gray mass that was gritty on cut section. Internally there were few areas of necrosis. Histologically, the specimen revealed sheets of pleomorphic spindle shaped cells, with strikingly organoid growth pattern, with insulae, ribbons, festoons, and trabeculae of tumor cells []. In many areas, cellular nests became detached from surrounding fibrovascular septa and foci of central geographic necrosis were present. Immuno-histochemistry of the specimen proved the diagnosis of neuroendocrine carcinoma of the thymus with tumor cells expressing cytokeratin positivity, strong synaptophysin positivity, chromogranin A positivity and TTF-1 positivity [Figures –]. Post-operatively, he received radiotherapy with 4000 rads followed by chemotherapy consisting of Cisplatinum, Bleomycin and Doxorubicin every three weeks for four cycles. But the patient continued to be symptomatic, and his condition worsened over a period of next three months and he died subsequently five months after the diagnosis due to the distant metastasis and recurrence of the disease. |
A 72-year-old female was admitted to hospital suffering from fatigue, weight loss and rectal bleeding. Total colonoscopy demonstrated adenocarcinoma of the sigmoid colon at 25 cm from the anal verge. A chest x-ray was normal. No sites of distant metastasis were reported on abdominal computed tomography (CT) scan. The serum carcinoembryonic antigen level was normal. The patient underwent sigmoidectomy in May 2008. During the operation, the left ovary was fixed at the site of the sigmoid colon cancer and was removed en block. Thorough macroscopic examination of the liver and rest of the abdomen showed no sign of metastatic disease. Histopathological examination of the specimen revealed a moderately differentiated mucus-producing adenocarcinoma, 3 cm in diameter located 5 cm from the peripheral surgical margin (figure ). The tumor invaded into but not beyond the muscularis propria (T2). Ki 67 antigen and p53 tumor suppressor protein staining were positive and epidermal growth factor receptor (EGFR) negative. The left ovary was free of neoplasmatic tissue. Only four lymph nodes were counted, free of metastatic adenocarcinoma. The patient had an uneventful recovery. On rectum examination one year later a palpable extramucosal mass was noticed at the anterior rectum wall. An abdominal CT scan revealed a tumor 2 cm in size at the lower anterior mesorectum in close relation with the posterior vaginal wall and a second mass 2 cm in size at the anterior abdominal wall midline (figure , ). Total colonoscopy showed no mucosal lesion. A chest x-ray was normal. Rectal endoscopic ultrasound (EUS) showed a tumor infiltrating the rectum muscularis propria from outside. Core needle biopsy demonstrated the presence of a mucus producing adenocarcinoma with the same histological futures with the primary tumor and therefore it was considered as metachronous metastasis. Serum carcinoembryonic antigen level was normal. Since no other site of recurrence was identified, an abdominoperineal resection was attempted []. At laparotomy, the anterior abdominal wall mass was located at the site of previous incision and after complete resection, fast biopsy showed adenocarcinoma. Surgical examination of the abdominal cavity showed no sign of reccurence. At that time, sinus bradycardia and ST segment depression was noticed on electrocardiogram (ECG) monitoring. The termination of the operation was decided and a loop transverse colostomy was immediately perfomed. Postoperative cardiologic examination revealed an acute muocardium infract and the patient was treated respectively. Chemo-radiation of the mesorectum tumor and re-evaluation for surgical excision was decided and she was discharged on the eleventh post operative day. |
Patient 1 was a 14-year-old male born prematurely at 27 weeks gestational age with non-shunted ventricular dilatation secondary to grade III–IV intraventricular hemorrhage. shows a T1 weighted anatomical image of patient #1 and three fiber tracts identified in the patient with AFQ; the right uncinate fasciculus, the right cortico-spinal tract and the forceps major of the corpus callosum. The child showed normal diffusion properties along the uncinate fasciculus, a tract that is spatially separated from the ventricles. The child has thinning of the corpus callosum and low FA along the full trajectory of the forceps major. This finding is consistent with other studies of children born prematurely. To test whether the reduced FA could be accounted for by partial voluming with CSF in this patient with enlarged ventricles we examined the patient's Tract Mean Diffusivity (MD) Profile. MD values were elevated at node 20, but otherwise MD values were within the normal range indicating that there was not a substantial change in the water content of forceps major voxels. Hence, we demonstrated that partial voluming with CSF could not explain the FA reduction.\nBy contrast, the left cortical-spinal tract had substantially increased FA throughout its trajectory compare to the control group. We interpret this increased FA as due to two major factors. The first relates to the tract itself. Ventricular dilatation may lead to stretching, displacement, and resulting increased coherence of the axons in the CST leading to increased FA . The second relates to crossing fibers. A distinctive feature of the left and right CST Tract FA Profiles of this patient is that FA increases near the superior portion where FA decreases for the healthy controls. In the typical subjects, this decrease in FA is the result of crossing fibers from the corpus callosum. In the patient, the amount of crossing fibers is most likely reduced, as indicated by the low callosal FA.\nThis case demonstrates that AFQ could be applied to a patient with extremely abnormal brain morphology. Tract Profiles provide novel insight into the neurobiology of this patient's white matter injury. |
A 32-year-old female patient presented with the complaint of a recently noticed spacing between the upper front teeth. (Figures –).\nThe patient noticed the spacing about 1 year before, after which she noticed it to be gradually increasing and associated with intermittent episodes of pus discharge which subsided on taking antibiotics as per advice at a local hospital. There were no associated complaints other than a cosmetic concern from the patient. There was no history of any previous dental treatment. Family history of similar complaints or early tooth loss could not be elicited. The patient was systemically healthy with no relevant medical history.\nThere were no abnormalities detected in extra oral examination except for a slightly tender and palpable left submandibular lymph node. Full complement of teeth was present. The oral hygiene status of the patient was good as revealed by the oral hygiene index. There was minimal amount of calculus and plaque. There was grade I mobility of 22, 31, 32, 21 and 22. Proximal contacts were lost between the teeth 14 and 13, 13 and 12, 21 and 22 and 22 and 23, 22 and 24 and between lower anterior teeth. There was labial migration and flaring of upper and lower anterior teeth with an evident distolabial migration of 22.\nGingival examination revealed normal color except for the labial aspect of 22 where it was slightly reddish. The margins were of knife-edge contour except for the labial aspect of 22 and 42 where it was bluntly rounded. The gingiva was firm and resilient except in the region on 22 where it was soft in consistency. There was no loss of stippling in the anterior regions. The position of the gingival margin was apical to the CEJ in the labial aspect of 22. There was generalized bleeding on probing, and exudation was present on the labial aspect of 22. All together there were minimal signs of inflammation other than bleeding on probing.\nA full-mouth periodontal charting revealed generalized periodontal pockets and clinical attachment loss ().\nPockets were especially deeper in the molar and incisor regions with slightly lesser involvement in the premolar region. The clinical attachment loss ranged from a maximum of 10 mm in the midpalatal aspect of 16 to a minimum of 2 mm in the premolar regions.\nAn OPG and full-mouth IOPA X-ray were performed which revealed the generalized distribution of alveolar bone loss which was a combination of both horizontal and vertical bone loss (). Routine blood examination results were within normal limits.\nBased on the history, examination findings, and the radiographic findings, a diagnosis of generalized aggressive periodontitis was made according to the criteria by AAP 1999 classification. |
A 22-year-old woman was admitted to our clinic with mild dyspnea. The patient had a history of cardiac disease in her bother, who had died. Additionally, she had an abortion due to cardiac abnormalities in the fetus. Physical examinations revealed cyanosis (oxygen saturation at room air = 85%), clubbing, and systolic murmurs in the mitral area (+2/6+) radiating to the anterior axillary line. Two-dimensional Doppler echocardiography demonstrated the enlargement of the right atrium and the normal size of tricuspid valve annulus; however, in the subcostal view, there was hypoplasia of the apical portion of the right ventricle while the sub-pulmonary outflow was normal. The systolic pulmonary artery pressure was 30 mm Hg. A redundant interatrial septum with a large atrial septal defect and a bidirectional shunt was illustrated, and there was also evidence of a small apical muscular ventricular septal defect with no significant left-to-right shunting ().\nCardiac magnetic resonance revealed a normal volume and function for the left ventricle, while a large atrial septal defect and a small ventricular defect were seen along with a localized interventricular bulging of the septum at the site of the ventricular septal defect (). According to the cardiac magnetic resonance results, the right ventricle volume was in the lower normal limit with a mildly reduced function ().\nCardiac catheterization was performed in order to delineate the right ventricle and pulmonary artery hemodynamics and pressures. The data obtained from the right ventricle angiogram showed a small right ventricle with apical hypoplasia. The pressure of the right atrium and right ventricular end-diastolic pressure was increased, and evidence of an atrial septal defect with right-to-left shunting was observed. The saturation and pressure data from right-heart catheterization and cardiac magnetic resonance results are depicted in and Table 2, respectively. Medical treatment with diuretics was done and subsequently, the patient was discharged. Re-evaluation was performed after 6 months by catheterization. The right ventricular end diastolic pressure and right atrium pressure decreased significantly, and the sizing balloon occlusion test showed no dramatic changes in the pressures. Accordingly, the atrial septal defect was closed with an Occlutech® device (21 mm) percutaneously. After 1 month, the patient had no symptoms and cyanosis and follow-up after 2 years showed no symptoms. |
34 years old patient, Gravida 6 Parity 3, previous 2 miscarriages (18 weeks & 12 weeks), was seen first at 23 weeks 4 days of pregnancy. She had undergone previous 3 cesarean sections and an evacuation of retained products of conception by curettage in 2013 for partial hydatidiform mole. At 27 weeks 5 days, she was admitted for vaginal bleeding. On further evaluation by ultrasound (), the diagnosis of placenta percreta was made (later confirmed by MRI). At 29 weeks, she had constipation with 2 episodes of urinary retention and she was put on continuous bladder drainage. She developed urinary tract infection and treated with appropriate antibiotics based on culture sensitivity. She continued to have repeated bouts of vaginal bleeding of varying amounts and severe constipation from 31 weeks of gestation.\nAt 32 weeks 4 days, patient underwent cystoscopy, which had shown signs of cystitis with no definite infiltration. She underwent classical cesarean section under combined anesthesia (Epidural + General). The umbilical cord was tied near insertion and the placenta was left in situ because there was no spontaneous separation. Then, the uterus was closed. Prophylactic temporary bilateral internal iliac artery balloons were inserted and inflated earlier. Uterine artery embolization was performed post cesarean section and selective angiograms confirmed adequate positioning. The patient required large volume of particles and still had incomplete embolization with the lower part of the uterus still showing some unblocked branches on both sides.\nPost-operatively, she was transferred to labor ward and within 4 hours, she developed clinical features of pulmonary embolism (PE). Some of her symptoms included drop in O2 saturation to 81%, tachycardia, chest pain, peripheral cyanosis, and signs of respiratory distress. Then, she was transferred to ICU and was initiated on heparin infusion. On chest X-ray, she had no atelectasis, pneumothorax, or pleural effusion. An immediate CT scan did not show any PE. There was no Doppler evidence of venous thrombosis in the femoral and popliteal venous systems. Later on day 1 post-operative, she had focal patchy consolidation left base and was started on parenteral meropenem, linezolid and fluconazole for the next 5 days. She had two consecutive CT scans on post-operative on days 2 and 3, which were negative. On ECG, there was right heart strain. She was now on enoxaparin. On the post-operative day 5, she was prescribed parenteral piperacillin-tazobactam for 5 days and she was shifted out of ICU next day. She had 500ml vaginal bleeding on the 9th post-operative day. 2 units PRBC were transfused. She was switched to oral cefuroxime and metronidazole and planned to continue on long-term low dose antibiotic. On post-operative day 11, she received methotrexate. On day 12, the MRA had shown the placenta was still enhancing with some areas of infarct and separation, fluid collection in the uterine cavity (present from day 1 post op, not increasing), with large ovarian veins, hugely distended and extensive pelvic varices, R>L, extensive collaterals. Her CRP was 12.7 mg/L.\nOn post-operative day 13, she underwent total abdominal hysterectomy. Intraoperatively, the bladder was densely adherent, drawn up, with large vessels in the broad ligament. The lower segment was bulging due to the presence of the placenta. The uterus was about 24 weeks’ size with adherent omentum. There was 100 mL of old blood in the cavity and the placenta was partially infarcted. The total blood loss was 2000 mL.\nPost-operatively, she was in ICU for 2 days receiving anticoagulation treatment (bridging treatment with enoxaparin + warfarin) and patient controlled analgesia. She had a bout of severe cough on day 4 and loose motions on day 5. She was diagnosed with vault hematoma, which was retro-vesical, about 120 ml in volume, treated conservatively. On day 10 she had been discharged from the hospital.\nShe presented to the ER on the post-operative day 16 and was diagnosed with chronic pulmonary embolism. Patient had a pulmonary embolus within the right middle lobe pulmonary artery; areas of sub-segmental embolus within the right lower lobe pulmonary arteries. She had no pleural effusions or consolidation and no mediastinal lymphadenopathy. She was readmitted for 4 days. She was started on therapeutic enoxaparin + warfarin. She was continued on 6 mg warfarin for 4 weeks after discharge. |
An 81-year-old Caucasian man had been diagnosed 5 years earlier with a gastric adenocarcinoma for which he underwent surgery. At the time, adjuvant radiotherapy was considered but was deferred when a liver metastasis was discovered. The patient was treated with palliative chemotherapy. He had an excellent treatment response and was subsequently followed without treatment for 2 years before undergoing radiofrequency ablation for progression of his hepatic lesion. After four cycles of second line palliative chemotherapy, the patient, still in excellent general condition, was referred for SBRT as consolidation treatment of what remained a metabolically active isolated liver metastasis. It was decided to proceed with treatment and platinum fiducials were chosen for SBRT targeting. Under local anesthesia, Nester embolization coils (Cook Medical Inc., Bloomington, IN, USA) were implanted using a 21-gauge needle under CT guidance. Each coil is 14 cm long, with a diameter of 0.889 mm and an approximate configuration of 11.7 loops creating an in vivo embolus of 4 mm.\nFor our patient, three coils were placed by an experienced interventional radiologist: the first on the anterior and medial border of the lesion, the second on the anterior lateral border and the third on the posterior border of the lesion. Before the placement of each coil, the location of the tip of the delivery needle was confirmed by CT imaging. On the immediate post-deployment CT, the third coil was not seen in the liver. A scout CT image localized it in the chest. During the procedure, the coil had unexpectedly migrated through the hepatic vein to the inferior vena cava (IVC) and lodged at the junction of the IVC and the right atrium. The patient was asymptomatic but was immediately referred to angiography for extraction of the coil.\nUsing ultrasound guidance and accessing through the right internal jugular vein, a guide wire was inserted over which a straight multi-side hole catheter was inserted and an angiogram was performed with the catheter tip within the right atrium (Figure ).\nUsing fluoroscopic guidance, an EN Snare Retrieval System (Hatch Medical L.L.C., Snellville, GA, USA) was used. The tip of the catheter was slightly curved and inserted over the guide wire toward the coil within the right atrium. The EN Snare was introduced through the catheter; it successfully grasped the coil, and the coil was removed through the sheath (Figure ). The patient was kept overnight for observation and no immediate or delayed complications were encountered due to the migration or retrieval of the coil.\nShortly after, it was possible to proceed with SBRT with daily image guidance based on the previously implanted platinum fiducials. Unfortunately, a short time thereafter, the patient developed intra-hepatic bile duct dilatation. This occurred secondary to documented tumor progression and was unrelated to the fiducial placement. The patient underwent percutaneous biliary drainage and was admitted to hospital for end of life care. |
34 years old patient, Gravida 6 Parity 3, previous 2 miscarriages (18 weeks & 12 weeks), was seen first at 23 weeks 4 days of pregnancy. She had undergone previous 3 cesarean sections and an evacuation of retained products of conception by curettage in 2013 for partial hydatidiform mole. At 27 weeks 5 days, she was admitted for vaginal bleeding. On further evaluation by ultrasound (), the diagnosis of placenta percreta was made (later confirmed by MRI). At 29 weeks, she had constipation with 2 episodes of urinary retention and she was put on continuous bladder drainage. She developed urinary tract infection and treated with appropriate antibiotics based on culture sensitivity. She continued to have repeated bouts of vaginal bleeding of varying amounts and severe constipation from 31 weeks of gestation.\nAt 32 weeks 4 days, patient underwent cystoscopy, which had shown signs of cystitis with no definite infiltration. She underwent classical cesarean section under combined anesthesia (Epidural + General). The umbilical cord was tied near insertion and the placenta was left in situ because there was no spontaneous separation. Then, the uterus was closed. Prophylactic temporary bilateral internal iliac artery balloons were inserted and inflated earlier. Uterine artery embolization was performed post cesarean section and selective angiograms confirmed adequate positioning. The patient required large volume of particles and still had incomplete embolization with the lower part of the uterus still showing some unblocked branches on both sides.\nPost-operatively, she was transferred to labor ward and within 4 hours, she developed clinical features of pulmonary embolism (PE). Some of her symptoms included drop in O2 saturation to 81%, tachycardia, chest pain, peripheral cyanosis, and signs of respiratory distress. Then, she was transferred to ICU and was initiated on heparin infusion. On chest X-ray, she had no atelectasis, pneumothorax, or pleural effusion. An immediate CT scan did not show any PE. There was no Doppler evidence of venous thrombosis in the femoral and popliteal venous systems. Later on day 1 post-operative, she had focal patchy consolidation left base and was started on parenteral meropenem, linezolid and fluconazole for the next 5 days. She had two consecutive CT scans on post-operative on days 2 and 3, which were negative. On ECG, there was right heart strain. She was now on enoxaparin. On the post-operative day 5, she was prescribed parenteral piperacillin-tazobactam for 5 days and she was shifted out of ICU next day. She had 500ml vaginal bleeding on the 9th post-operative day. 2 units PRBC were transfused. She was switched to oral cefuroxime and metronidazole and planned to continue on long-term low dose antibiotic. On post-operative day 11, she received methotrexate. On day 12, the MRA had shown the placenta was still enhancing with some areas of infarct and separation, fluid collection in the uterine cavity (present from day 1 post op, not increasing), with large ovarian veins, hugely distended and extensive pelvic varices, R>L, extensive collaterals. Her CRP was 12.7 mg/L.\nOn post-operative day 13, she underwent total abdominal hysterectomy. Intraoperatively, the bladder was densely adherent, drawn up, with large vessels in the broad ligament. The lower segment was bulging due to the presence of the placenta. The uterus was about 24 weeks’ size with adherent omentum. There was 100 mL of old blood in the cavity and the placenta was partially infarcted. The total blood loss was 2000 mL.\nPost-operatively, she was in ICU for 2 days receiving anticoagulation treatment (bridging treatment with enoxaparin + warfarin) and patient controlled analgesia. She had a bout of severe cough on day 4 and loose motions on day 5. She was diagnosed with vault hematoma, which was retro-vesical, about 120 ml in volume, treated conservatively. On day 10 she had been discharged from the hospital.\nShe presented to the ER on the post-operative day 16 and was diagnosed with chronic pulmonary embolism. Patient had a pulmonary embolus within the right middle lobe pulmonary artery; areas of sub-segmental embolus within the right lower lobe pulmonary arteries. She had no pleural effusions or consolidation and no mediastinal lymphadenopathy. She was readmitted for 4 days. She was started on therapeutic enoxaparin + warfarin. She was continued on 6 mg warfarin for 4 weeks after discharge. |
A 4-year-old girl, who weighted 13 kg, presented to our department for a scheduled follow-up for the transcatheter closure of a multiple secundum ASD. She also had a history of a transcatheter closure of a large patent ductus arteriosus at the age of 10 months, because of congestive heart failure. A transthoracic echocardiographic examination revealed two atrial septal defects measuring 12 and 4 mm, respectively, thatwere 5 mm apart. The total septum was 32 mm in length and had adequate surrounding rims []. In addition, a dilated right ventricle and tricuspid valve regurgitation were present, and no remarkable findings were observed in the other investigations.\nCardiac catheterization was performed under general anesthesia using transesophageal echocardiography control. We originally intended to place a 5-French (Fr) sheath in the right femoral vein and the right femoral artery for hemodynamic measurements and monitoring. However, we could not pass the guidewire into the inferior vena cava. Manual contrast injection was performed in the femoral vein, and an obstruction in the inferior vena cava was observed and identified as a thrombus. Catheterization was continued by using the right internal jugular venous access with a 5 Fr sheath. Heparin 100 units per kilogram of body weight was administered, and a 5 Fr Judkins right catheter with a hydrophilic guidewire was introduced, under transesophageal echocardiographic control through the larger defect into the left atrium and the left lower pulmonary vein instead of the left upper pulmonary vein. A manually curved, stiff exchange guidewire then replaced the catheter in that position, over which a 7F long sheath and a 16 mm Amplatzer septal occluder (St. Jude Medical, Inc.; Plymouth, MN, USA) device were glided into the left atrium. The left and right device discs were opened sequentially under fluoroscopic and transesophageal echocardiographic guidance, and a successful deployment of the device was accomplished after several and repeated trials, aiming for an ideal device orientation []. Transesophageal echocardiography revealed no residual shunts, normal atrioventricular valve function, and normal superior and inferior vena cava blood flow. The next day, a transthoracic echocardiographic examination was normal. Aspirin therapy was started with a six-month course, and a follow-up was planned for the patient. The coagulation profile tests were normal, and the patient was discharged the next day. |
In September 2000, a 35-year-old female without any history or clinical stigmata of NF-1 presented to her primary physician with complaints of a dull, localized left upper leg pain of several months' duration. An abdominal mass was palpated during a physical examination. Magnetic resonance imaging (MRI) as well as a computed tomographic (CT) scan of the abdomen and pelvis showed a large well-defined, near spherical mass in the left false pelvis which enhanced heterogeneously at a mean Hounsfield value of 44 units (Figure ). The mass displaced the external iliac vein medially and psoas muscle laterally. It also abutted the upper surface of the left ovary without truly invading any of these or other surrounding structures. However, the mass was believed to be in the course of the left genitofemoral nerve and lumbar plexus. The decision was made to resect the mass. Intraoperatively, the tumor's capsule was found to be densely adhered medially to the external iliac vessels, with at least 10 external venous branches directly supplying the tumor. The tumor was carefully marsupialized out of the retroperitoneal area and the decision was made to leave the residual capsule, since an attempt at its removal would have entailed a highly morbid procedure that was not felt to be justified based on the histopathologic appearance of the tumor on frozen sections. Intraoperatively, a pigmented macular lesion with faintly irregular edges was noted in the left upper thigh, which was biopsied. Pathologic examination showed a malignant melanoma with spitzoid features. The precise circumstances regarding the duration of the lesion and whether there had been any increase in its size was unclear. She subsequently underwent a wide local excision (4 × 12 cm skin ellipse was removed) and sentinel lymph node biopsy, both of which showed no residual melanoma. The patient's postoperative course over the subsequent 2 years was remarkable for a relatively slow but progressive improvements in the neurologic symptoms related to her surgery. However, she showed no evidence of either tumor recurrence at last follow-up, 26 months postoperatively. |
An 82-year-old, white woman with a history of alcohol abuse, presented to the accident and emergency department with a 4-hour history of an acutely ischaemic right upper limb with motor and sensory deficit. A hard tender, pulsatile mass was palpable in the right subclavian area with significant bruising; there was a palpable right subclavian pulse with no pulses distal to this. X-ray revealed a fracture of the surgical neck of the right humerus with the humeral head abducted and externally rotated, while the humeral shaft was displaced medially (Fig. ).\nTen weeks previously, she had presented with a fracture of the surgical neck of the right humerus following a fall whilst under the influence of alcohol. On that occasion, sensory and motor function of the limb had been recorded to be fully intact by the medical staff in Accident and Emergency and there had been a full complement of pulses. Given she had no neuro-vascular deficit in the affected limb, the vascular surgeons were not involved initially. Under guidance of the orthopaedic surgeons, she had been treated conservatively with a collar and cuff due to her age and history of current alcohol abuse. She was to have been followed up fortnightly in the orthopaedic fracture clinic – but failed to attend after her second visit. She had no neuro-vascular deficit on follow-up. She denied any further falls or trauma to the right upper limb.\nThe acute nature of the current presentation together with neurological compromise prompted classification as category-II acute limb ischaemia (Society for Vascular Surgery/International Society for Cardiovascular Surgery classification) [] and urgent angiography was performed with a view to revascularisation. This revealed a pseudoaneurysm of the third part of the right axillary artery with complete occlusion of the right brachial artery distal to this (Fig. ).\nOperative treatment was undertaken with initial exposure and control of the subclavian artery above the clavicle (Fig. ). Simultaneous exposure of the brachial artery in the antecubital fossa was performed and a size 3 Fogarty embolectomy catheter passed distally down the brachial artery. Both radial and ulnar arteries were found to contain thrombus which was cleared with good back flow. The proximal brachial and distal subclavian arteries were ligated in continuity. Two interconnected Javid™ shunts were inserted to carry blood flow from the subclavian to the brachial artery in order to maintain perfusion (Fig. ) during open reduction and internal fixation of the fractured humerus, after which a subclavian to brachial bypass was performed using reversed long saphenous vein. The fracture was temporarily stabilised using external splints to immobilize the limb whilst securing vascular continuity.\nPostoperatively, the patient had strong radial and ulnar pulses with complete resolution of her motor and sensory dysfunction within 72 hours. Her postoperative course was uncomplicated and she was discharged on the 10th postoperative day. Early postoperative duplex scan performed at 6 weeks revealed satisfactory function of the vein graft. |
We present the case of a 77-year-old ambulatory man with hypertension, sarcoidosis, complete atrioventricular block status post-pacemaker implantation, chronic kidney disease due to FSGS, and right facial nerve paralysis, who presented with sporadic gait and right face numbness. He was diagnosed with sarcoidosis by biopsy of a tumor in front of the right tibia 14 years before presentation. Since the tumor and abdominal lymphadenopathy were the only manifestation of sarcoidosis and no other signs of organ involvement were present, he received no immunosuppressive treatment. The abdominal lymphadenopathy had been stable over time. Nine years before presentation, he was referred to our nephrology clinic to determine the cause of chronic kidney disease. His serum creatinine level was 1.2 mg/dL and he had proteinuria of 0.4 g per day. Hematuria was not present. Renal biopsy revealed six globally sclerotic glomeruli among all 34 glomeruli (18%) and some residual glomeruli with segmental sclerosing lesions, but no involvement of sarcoidosis. He was diagnosed with primary FSGS. Since the proteinuria was mild, he did not receive immunosuppressive treatment.\nOne year after that, the patient experienced palpitations and was diagnosed with complete atrioventricular block. Coronary angiography showed no significant stenosis of the coronary arteries, and he underwent pacemaker implantation. Whether sarcoidosis contributed to the complete atrioventricular block was unclear. The abdominal lymphadenopathy and the dyskinesia of the ventricular septum were stable and did not progress over time.\nThe patient was stable for eight years, until when he started to suffer from sporadic gait and right face numbness that occurred and resolved within a day every few weeks. Three months later, the symptoms recurred along with sudden dysarthria and left limbs weakness. Physical findings were notable for pronator drift on the left side. Perfusion computed tomography (CT) with iodinated contrast and CT angiography revealed no ischemic lesions or occlusion of major cerebral arteries. The symptoms disappeared three hours after the onset. A transient ischemic attack (TIA) was suspected, and he was admitted to the stroke unit. Ultrasonography revealed no stenosis of the internal carotid arteries, and transesophageal echocardiogram showed no abnormalities of the atrial septum. His pacemaker detected paroxysmal atrial fibrillation, which was presumed to be the etiology of the TIA. Thus, edoxaban 30 mg per day was started and he was discharged after one week of hospitalization.\nOne month after his discharge, his left leg started to swell and his gait worsened. Urinary protein excretion was 0.6 g per day, serum creatinine was at the baseline level of 1.6 mg/dL, and serum albumin level was 3.8 g/dL. Although no coagulopathy was found, ultrasonography revealed left femoral vein thrombosis that was 41 mm long. Edoxaban was stopped, and heparin was administered intravenously for two weeks. Low mobility due to his gait was presumed to be the cause of development of deep vein thrombosis (DVT). The patient was switched to warfarin and was discharged, but the left leg edema persisted. Three months later, he developed complications of urinary retention and constipation.\nFour months after discharge, the patient presented to the emergency department with sudden left leg pain and inability to walk. The entire left lower limb was slightly pale and had slow pitting edema. The left dorsal artery was not palpable, and the left femoral artery was barely palpable. Contrast CT revealed occlusion of the left femoral and superficial femoral arteries together with the known DVT in the left femoral vein (Fig. , ). Emergency thrombectomy for acute arterial occlusion was performed and the leg perfusion resumed. The emboli (maximum of 23 mm in diameter) were sent for pathological examination. The patient was admitted to the hospital and started on heparin infusion in place of oral warfarin. The history of recent TIA implied hypercoagulable state, but again no coagulopathy was found. While malignancy screening was being planned, the pathology of the arterial emboli revealed an unusual and surprising finding: the surface of the thrombi was filled with large atypical lymphoid cells (Fig. ) and was covering the necrotic interior of the thrombi. Immunohistochemical analysis showed that the tumor cells on the surface and the necrotic interior of the thrombi were positive for CD20 and CD79a but negative for CD3 (Fig. , ), which is characteristic of B cells. Leukocytosis was absent (white blood cell, 4,000/μL; segmented neutrophil, 55%; lymphocyte, 34%; monocyte, 9%; eosinophil, 2%). Serum soluble interleukin-2 receptor level was 1,548 U/mL (normal, 122–496 U/mL); lactate dehydrogenase (LDH) level, 808 U/L (normal, 120–245 U/L); LDH-2 fraction, 39% (normal, 28–35%), and LDH-3 fraction, 32% (normal, 21–27%). These findings were consistent with large B-cell lymphoma with intravascular proliferation, but the etiology of the aortic thrombi was unclear.\nThe hematology consultation team considered that the patient needed further biopsy to determine the etiology. Bone marrow biopsy showed normocellular marrow with normal maturation, but with infiltration of CD79a-positive large atypical lymphoid cells within the small vessels (Fig. , ). Although no lymphadenopathy was detected on palpation, CT scan showed swollen bilateral axillary and inguinal lymph nodes, which were up to 30 mm in diameter. While surgical biopsy of the right axillary lymph node and random skin biopsy were planned for diagnosis, the patient developed a complication of sepsis presumably due to pyelonephritis on hospital day nine. Piperacillin/tazobactam and vancomycin were started. Because partial thromboplastin time was prolonged, biopsies were withheld. Although white blood cell and neutrophil counts were improving, the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. The patient had a do-not-resuscitate order. His family agreed to an autopsy. |
A 47-year-old woman visited our hospital in January 2003 because of a painful right breast mass with skin dimpling. On initial evaluation, chest CT revealed the presence of a heterogeneous enhancing breast mass measuring 9 cm with skin invasion and multiple conglomerated lymph nodes in the right axillary area. In addition, multiple metastatic pleural masses with malignant pleural effusion and mediastinal lymph node enlargement were also observed. A bone scan showed increased uptake in the sternum and right sixth anterior rib. There was no evidence of liver metastases on the initial CT scan (). Fine-needle aspiration cytology of the breast mass showed atypical malignant cells. Accordingly, she was diagnosed with stage IV right breast cancer with multiple pleural and bone metastases. She subsequently received palliative chemotherapy with docetaxel and epirubicin. Follow-up CT scan performed after four cycles of chemotherapy showed partial regression of the breast mass and multiple metastatic masses in the pleura and axillary area. She then underwent palliative total mastectomy of her right breast because of an ulcerated skin lesion. Pathological examination showed invasive ductal carcinoma with nuclear grade 2 and lymphatic and perineural invasion. Immunohistochemistry studies showed positive staining for the estrogen receptor (ER) protein, progesterone receptor protein, and human epidermal growth factor receptor 2 (HER2) (score 3). She received two additional cycles of chemotherapy with docetaxel and epirubicin. A follow-up CT scan showed stable disease and she then started taking tamoxifen (20 mg daily) in July 2003.\nTwo years later, in November 2005, a surveillance breast ultrasound showed an irregular circumscribed mass measuring 1 cm on theright mastectomy site. The patient underwent a wide local excision and pathology confirmed an invasive ductal carcinoma. Although previous metastatic lesions showed stable disease, a new chest wall lesion had developed; therefore, her treatment was switched from tamoxifen to the non-steroidal aromatase inhibitor anastrozole. At that time, she was postmenopausal, based on her serum follicle-stimulating hormone levels. In January 2007, a bone scan showed new increased uptake in the right second rib, the third anterior rib, and the right acetabulum. She had progressive disease of the bone; therefore, capecitabine (an oral prodrug of 5-fluorouracil; 2,500 mg/m2/day) was started (two weeks on, one week off). After nine cycles of chemotherapy, she had stable disease and was off chemotherapy for approximately four years with no evidence of progression.\nHowever, follow-up CT scan performed in June 2011 showed multiple newly developed peripheral enhancing nodules in the liver (). She received six cycles of chemotherapy until November 2011, and CT scan showed that her metastatic hepatic lesions were stable. Since then, her chemotherapy has been discontinued because of grade 4 neutropenia and osteomyelitis of the mandible. Four months later, follow-up CT scan showed ill-defined heterogeneous enhancing lesions in the entire liver with surface nodularity. The liver had a nodular contour consistent with cirrhosis, as well as moderate ascites (). F18 fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET-CT) also showed disseminated and innumerable lesions throughout almost the entire liver with increased FDG uptake (maximum standardized uptake value [SUVmax], 7.3), suggesting diffuse liver metastasis (). Palliative chemotherapy with docetaxel and epirubicin was started again. Endoscopy showed grade 3 large esophageal varices with a red color sign. She denied a history of liver disease, alcohol use, and risk factors for viral hepatitis. Serologies for viral or autoimmune etiologies of cirrhosis were negative and the serum level of alpha-fetoprotein was normal. An ultrasound-guided liver biopsy was performed in order to confirm the cause of the cirrhotic changes. Pathological examination showed that the hepatic parenchyma was diffusely infiltrated by poorly differentiated carcinoma cells. Hepatocytes were almost replaced by carcinoma cells and extensive fibrosis between clusters of cancer cells was observed (). Immunohistochemical staining was positive for gross cystic disease fluid protein-15, MOC 31, ER, and HER2 (score 3), but negative for hepatocytes (). Follow-up PET-CT showed progression of liver metastases with increased FDG uptake (SUVmax, 9.0) since the scan performed in June 2011. Therefore, we concluded that the hepatic metastases from breast cancer had progressed and led to cirrhotic changes in the liver.\nSubsequently, her chemotherapy regimen was changed to trastuzumab and docetaxel. However, bleeding of esophageal varices occurred on the sixth day after initiation of chemotherapy, and hepatic failure progressed gradually. Therefore, the patient decided to discontinue chemotherapy. |
A 37-yr-old female presented to a private spine clinic with a chief complaint of episodic neck and back pain. The subject began care while her daughter was being treated for scoliosis in the same clinic. She presented with a past medical history including previous diagnosis and treatment for adolescent idiopathic scoliosis. Her previous treatment included spinal fusion and Harrington rod instrumentation. Preoperatively, a 58° right thoracic scoliosis was found between T6 and T11. Harrington rod instrumentation reduced the scoliosis to 26°. We were unable to review her medical records pre and post arthrodesis. Although her family history identified a possible genetic component with her daughter's medical history, her preceding family history was negative for scoliosis.\nThe subject initially filled out a Functional Rating Index. This index, described and tested by Feise et al [], is a combination of the Neck Disability Index and the Oswestry Back Pain Index. This form provides a valid and reliable self-rated assessment of functional improvement in daily activities.\nOn static visual posture examination, a moderate anterior right shoulder, a protruding right scapula, and a right rib hump were identified. These visual postural findings are used as screening indicators so that unnecessary radiographic studies are not undertaken. Adam's test confirmed the right rib hump on forward bending. This test is classically used in the primary care setting to screen for scoliosis, although its reliability has been called into question [].\nThe radiographs series consisted of lateral cervical and lumbar views, as well as opposing frontal views. The lateral films were taken to calculate the amount of cervical lordosis, forward head posture, and lumbar lordosis. The cervical lordosis was measured from an angle between 2 lines intersecting the posterior C2 and C7 vertebral bodies. The lumbar lordosis was taken from the angle formed by the intersection of 2 posterior tangent lines drawn from the back of L1 and L5. Preliminary evidence suggests that correcting the sagittal spine before reducing the scoliotic curvature may promote a longer lasting correction [,]. In this case, the initial cervical lordosis measured 23° from C2 to C7, the initial forward head posture measured 31 mm, and the lumbar lordosis measured 31°. Analysis of forward head posture was performed by drawing a vertical line from the posterior inferior corner of C7 upward []. The distance from this line to the posterior superior corner of C2 is measured in millimeters. The initial standing AP radiograph showed a right thoracic scoliosis of 35°, shown in Figure . This measurement was taken from a Cobb angle drawn between the superior endplate of T6 and the inferior endplate of T11. We used a sectional view of the thoracolumbar spine to reduce positional distortion commonly encountered on full-spine films []. The film was taken at a 72" film to focal distance (FFD) to reduce magnification distortion. For radiographic analytical purposes, we used the positioning and analysis methods outlined by Harrison et al [,-]. These methods have shown good to excellent reliability in terms of patient positioning, and inter- and intra-examiner reliability. Initially, the patient self-rated her back and neck pain as a 7/10 on a numerical pain rating scale.\nThe Pettibon corrective procedures [] were used in this patient's care plan. The goal of these procedures is to promote a normal [-] sagittal spinal contour. A specific treatment plan was created based upon a trial treatment involving the Pettibon procedures. The patient received bilateral cervical spine traction-type manipulation to mobilize several cervical spinal joints, and then was immediately fitted with a 4-lb Pettibon Headweight. ® The patient walked on a treadmill for 10 minutes while wearing the headweight. After 10 minutes, a follow-up lateral cervical radiograph was taken while wearing the headweight. The purpose of this lateral stress view is to evaluate the potential improvement in cervical lordosis and reduction in forward head posture. Cervical lordosis and forward head posture are again measured on these stress views to evaluate response to treatment. Although earlier studies suggest that a 23° cervical lordosis may also be normal [-], newer research identifies a cervical lordosis closer to the 40° range [,,] Despite this evidence; the concept of a normal cervical lordosis remains a debatable issue. Once it was determined that the patient could benefit by the proposed treatment, a plan was developed and implemented specifically for her. Her plan included once-weekly office visits, with an emphasis on home care exercises to promote patient independence. Each visit consisted of warm-up procedures, manipulation, and rehabilitative exercises.\nThe warm-up procedures consisted of Pettibon Wobble Chair® Exercises, shown in Figure . The Pettibon Wobble Chair® is a chair designed to isolate the lumbar spine so that core training may take place. The goals of the chair are to promote lumbar stability, muscular coordination, and increase flexibility. However, the benefits of the chair itself remain to be investigated. The Wobble Chair® exercises are performed by holding the head and shoulders still, moving only the pelvic girdle. The exercises consist of a front-to-back motion, a side-to-side motion, and clockwise/counterclockwise circles. Each exercise was performed 20 times, for a total of 80 repetitions at each office visit.\nSide-posture lumbopelvic adjustments were delivered bilaterally to mobilize the sacroiliac joints. Cervical spine manipulation was performed by hand in accordance with the radiographic findings. The cervical spine manipulative procedures can be found in the osteopathic literature [].\nThe rehabilitative included the use of a 4-lb anterior Pettibon Headweight®, a right low shoulderweight, and a left high shoulderweight. An illustration of the weighting system is shown in Figure . During each office visit, the subject wore the headweight and shoulderweights while standing or walking. This exercise was performed for 10 minutes following the manipulative procedures. The patient was instructed to wear the headweight and shoulderweights at home for 20 minutes twice daily. Positional traction, on 2 triangular foam blocks placed at the cervicothoracic and thoracolumbar junctions, was performed once daily immediately before bed for 20 minutes.\nAfter 8 visits in 8 weeks, post radiographs were taken to quantify changes in the sagittal and frontal spinal curves. Additionally, the subject filled out a follow-up Functional Rating Index to compare to the original. The Functional Rating Index score dropped from a 33% disability rating to 8%, and the numerical pain rating scale, rated a 7.0/10 at the onset of care, dropped to a 0/10. The average numerical pain rating scale score over the 8-week span was 3.3 out of 10.\nOn the post-treatment anteroposterior radiograph, the Cobb angle from T6–T11 was reduced from 35° to 22°. Her cervical lordosis measured 40°, while her forward head posture reduced to 13 mm. The follow-up radiographs were taken at the beginning of the 9th visit prior to treatment, one week after the previous treatment.\nA 30-yr-old African-American male presented to a private spine clinic with a chief complaint of chronic mid thoracic pain. The patient had a previous medical diagnosis of Scheuermann's Disease. Moderate wedging was found on previous lateral lumbar and thoracic radiographs at the levels of T7–T10. The patient reported having the back pain consistently over the last 8–10 years, with recurrent episodes of intense myospasms occurring in the paraspinal musculature at the thoracolumbar junction. The patient had been previously managed unsuccessfully with prescription NSAIDS, muscle relaxants, and physical therapy consisting of cryotherapy, electric stimulation, and postural isotonic exercises. The patient could not recall any childhood traumatic events that may have contributed to the vertebral wedging asymmetry.\nThe subject initially filled out a Functional Rating Index []. We used this form to provide an objective assessment of functional improvement in daily activities. On static visual posture examination, a moderate high and anterior left shoulder and a right rib hump were identified. The paraspinal thoracolumbar musculature had also been significantly developed. Although these factors are not differential for Scheuermann's Disease, they do represent postural abnormalities often associated with scoliosis. Palpatory findings included marked areas of spasticity over the right latissimus dorsi, the left trapezius, the left quadratus lumborum, and the left rhomboid muscles.\nStanding anteroposterior and lateral cervical and lumbar radiographs were obtained and analyzed for regional alignment as previously described. Gross radiographic visualization showed a postural swayback positioning, where the pelvis shifts anterior in relation to the thoracic cage. This may result from activation of the pelvo-ocular reflex to compensate for a forward head position []. The initial absolute rotation angles (ARA) from C2–C7 on the lateral cervical view [] and L1–L5 on the lateral lumbar view [] were drawn and measured. Prior to treatment, these angles measured 32° and 55°, respectively. According to Harrison et al, the normal lumbar lordosis should measure 39.7°, with a majority of the lordosis comprised in the L4-S1 region []. Prior to treatment, the forward head posture measured 22 mm, compared to an average normal of <20 mm []. The vertical axis line (VAL), measured from the anterior portion of the sacral base, should intersect the T11/T12 area []. In this case, the patient's VAL was 56 mm anterior to this interspace, consistent with a swayback type of posture. In the coronal views, a left thoracic scoliosis was found between the levels of T1–T5 measuring 22°. Nothing remarkable was found on the AP lumbopelvic.\nThe patient began an initial treatment plan consisting of 3 weekly visits for 4 weeks. The goals of this initial treatment plan were very specific, including restoring normal sagittal cervical and lumbar curves, reducing forward head posture, and reducing the swayback posture.\nThe initial 4 weeks of care consisted of manipulative and rehabilitative therapy designed to improve the static alignment of the sagittal spine. These methods are part of the Pettibon system []. The first 12 visits entailed the same procedures in the same order. To begin each visit, the patient performed a series of exercises on a Pettibon Wobble Chair®. This chair is consists of a multiplanar seat that allows the user to perform specific spinopelvic motion patterns. Clinical observation by the authors suggests that these exercises seem to make the manipulative treatment easier on the patient.\nIn this case, manipulative treatment included bilateral cervical manipulation and anterior thoracic manipulation to mobilize any restricted cervical and/or thoracic segments.\nFollowing the manipulative treatment, the patient was fitted with a Pettibon Headweight® containing 4 lbs on the front of the forehead. The patient walked for 15 minutes while wearing the headweight. After 15 minutes, the patient laid supine on a pair of high-density foam blocks to promote a normal sagittal spinal contour. This was done while lying on an intersegmental traction table for 7 minutes. The patient was prescribed specific home care exercises to be performed daily between visits, and was instructed to walk with the Pettibon Headweight® for 20 minutes twice daily on non-clinic days, and lie on the high-density foam blocks for 20 minutes every night immediately before bed. After 4 weeks, post treatment lateral cervical and lateral lumbar radiographs were taken to quantify improvement in sagittal alignment.\nThe post lateral cervical showed a 32° cervical lordosis and 5 mm of forward head posture. The post lateral lumbar showed a 44° lumbar lordosis, while the vertical axis line fell 30 mm from the T11/T12 interspace. The 4-week functional rating index improved from a 70% disability to 50% disability, while the numerical pain rating scale dropped from a 9/10 to an 8/10.\nGiven the presence of bony deformity, we felt that significant time must be spent reducing the asymmetrical loading in the thoracic spine for coronal correction to be achieved. Therefore, the frequency of visits remained at 3 times per week over the next 20 weeks. Over this 20-week period of care, the manipulative treatment remained the same. However, several new rehab procedures were added. The patient still wore the headweight for 15 minutes immediately following the manipulative treatment. After the headweight, the patient worked out on the Pettibon Wobble Chair® while simultaneously performing cephalad traction, demonstrated in Figure . Following this procedure, a specific isometric exercise was performed on a Pettibon Linked Trainer. ® This exercise, shown in Figure , is designed to isolate the right rhomboid muscle. Theoretically, the linked trainer stabilizes the scapula, thereby functionally changing the origin and insertion of the rhomboid. This form of exercise has been previously illustrated with practitioner assistance []. Typically, the function of the rhomboid is to retract the scapula. However, when the scapula is stabilized, now the muscle may effectively pull on its proximal attachment, that being the spinous processes from T5–T8. Therefore, by switching the action of the muscle, our goal was to use the rhomboid to help reduce the left thoracic scoliosis. The patient was instructed to perform this exercise by pulling and holding for 10 seconds, repeating this process until the muscle is sufficiently fatigued. Finally, lateral traction was performed on the thoracic scoliosis using a high-density foam block while in a side-lying position. This block was placed beneath the apex of the scoliotic curvature for 15 minutes. Home care exercises remained the same. However, the frequency of the exercises was dropped to 3 times per week instead of daily. At the conclusion of the 20 weeks, post treatment AP cervicothoracic and lumbopelvic radiographs were taken to quantify improvement. The Cobb angle of the left thoracic scoliosis from T1–T5 reduced to 14°. A comparative view of the pre and post AP cervicothoracic views is shown in Figure . A 20-week functional rating index score dropped to a 28% disability rating, while the numerical pain rating scale dropped to a 6/10.\nA 23-year-old female presented with bilateral diffuse neck and lumbodorsal pain, and right-sided scapular and shoulder pain. The pain was constant and sharp in nature with radicular pain into the right arm and elbow. At age 12, her primary care physician diagnosed her with adolescent idiopathic scoliosis. At that time, the treatment plan was mainly comprised of observational methods, such as radiographs, visualization, and MRI. About one year before presenting to the primary author's clinic, she was referred for physical therapy by an orthopedic surgeon, which produced little subjective benefit, according to the patient.\nOn visual examination, a prominent left posterior rib hump was identified. In the frontal plane, she also displayed a marked high left shoulder with anterior rotation. Left anterior pelvic rotation was also well visualized. Given these preliminary findings, along with the positive past history of scoliosis, radiographic imaging was ordered to locate and calculate the nature and severity of the scoliosis. Initial standing 14" × 17" sectional radiographs showed a 37° left thoracic scoliosis, measured from the superior endplate of the T3 vertebra and the inferior endplate of the T7 vertebra. She also had a 26° right lumbar scoliosis measured from the superior endplate of T10 and the inferior endplate of L3. In the sagittal plane, her initial cervical lordosis measured 18°, while her lumbar lordosis measured 50°.\nThe patient began a treatment plan of 3 visits per week for 4 weeks, followed by once weekly visits for 12 weeks. Goals for the first 4 weeks of treatment included: 1) improvement of sagittal spine alignment, 2) reduction in pain and symptoms, and 3) functional improvement. A specific treatment routine was followed at each visit for the first 12 visits.\nEach visit began with spinal warm-up exercises performed on a Pettibon Wobble Chair™. The patient then received a brief (less than 15 minutes) session of deep tissue massage therapy applied to the postural muscles. Following these procedures, manipulative intervention took place. The manipulative techniques are collectively taught within the Pettibon technique [], and were employed according to this methodology. First, a posteroanterior high-velocity, low amplitude (HVLA) procedure was applied to mobilize the thoracolumbar region. This was followed by anterior thoracic manipulation to mobilize the cervicothoracic region. A side-lying sacral manipulation was performed bilaterally to mobilize the sacroiliac joints and the lumbosacral joint. Cervical manipulation was performed only on those visits where a supine leg check revealed evidence of leg length inequality (LLI). In the cervical region, an HVLA thrust was applied cranially, thus creating a traction-type adjustive force compared to more traditional shear- or rotary-type cervical manipulative procedures. All of the manipulative techniques are well illustrated and explained by Gibbons and Tehan []. The patient received cervical manipulation in 8 of the first 12 visits.\nImmediately following the manipulative intervention, the patient performed her spinal rehabilitative care. In her case, a 4-lb Pettibon Headweight was worn on the front of the head for 10 minutes while maintaining a standing position. Finally, the patient ended each of these visits with the supine positional traction for 7 minutes. The patient was instructed to perform the headweight twice daily between visits for 20-minute intervals. She was also given a set of foam blocks to lie on at night for 20 minutes immediately before bedtime.\nAfter this initial 4-week treatment period, a follow-up radiographic series was obtained, along with a follow-up Functional Rating Index. Comparative radiographic analysis showed a reduced Cobb angle of 29° from T3–T7 and 18° from T10-L3. The sagittal cervical lordosis improved to 32°, while the lumbar lordosis decreased to 45°. The follow-up Functional Rating Index score dropped from 48% to 28% disability.\nFollowing this treatment period, clinical visits dropped to once weekly over the next 12 weeks. During this time, the Pettibon Linked Trainer™ was incorporated into her treatment plan. The Linked Trainer™ exercises were performed after the anterior headweighting procedure at each visit. Dynamic cervical traction was also applied while performing the Pettibon Wobble Chair™ exercises, immediately prior to the spinal manipulative therapy. Finally, a side-lying traction procedure was added to her treatment to help lengthen the soft tissue structures on the concave side of the spinal curvatures. A triangular foam block was placed under the patient's left side, below the apex of the thoracic curvature, while a 25-lb weight was placed above the apex of the lumbar curvature. The patient assumed a left side-lying position during this traction session. This traction maneuver followed the anterior headweighting and the Linked Trainer™ exercises. This procedure was performed for 40 minutes at each office visit as well as at home once daily. The frequency of headweight use at home dropped to 3 days weekly instead of daily.\nAfter 12 weeks of the foregoing treatment, the patient was again re-evaluated using static spinal radiography and the Functional Rating Index. Radiographic analysis demonstrated a 21° left thoracic scoliosis from T3–T7, and a 15° right lumbar scoliosis from T10-L3. Her Functional Rating Index score further reduced to an 18% disability. The patient was asked to continue once daily home treatment consisting of the side-lying traction procedure for 40 minutes, and supine positional traction 20 minutes immediately before bedtime. She was also instructed to continue wearing the anterior headweight at home 3 days a week for 15 minutes per day. After 10 months under this home care regimen, the patient presented for a second follow-up evaluation. At this time, her Functional Rating Index reduced to an 8% disability, while her sagittal cervical and lumbar curves marginally improved to 34° and 42°, respectively. Her left thoracic scoliosis was further reduced to 18°, and her right lumbar scoliosis was maintained at 15°. Therefore, after a total of 4 months of active treatment and 10 months of weekly home care rehabilitation, her spinal curvatures were reduced a total of 19° in the thoracic curvature and 21° in the lumbar curvature. Her pre- and post- radiographs are shown in Figure . |
A 36 years old Saudi Male was brought to the Emergency Room following a generalized convulsions and loss of consciousness. He was driving a car and felt an acute sharp pain at right shoulder, few seconds later, he developed blurring of vision and he was able to stop the car and lost consciousness for 15–20 minutes. He was observed to have rolling up of the eyes with frothy salivations and trauma to the tongue. The patient complained of severe arthralgia and pain on minimal movement of any joint. On examination he was noted to have petechiae all over his body, no signs of meningeal irritation and there was a lesion over the scapula which was diagnosed as a snake bite. Blood investigations were normal and computerized tomography of the brain was normal. Patient was loaded with phenytoin 25 mg/Kg body weight. In the ward he developed echymotic lesions on the skin and upper extremities, with recurrent vomiting, distended abdomen, paralytic ileus, and deteriorating renal and liver function tests. In the intensive care unit the patient was treated by antivenom and supportive therapy. At the end of the 7th day the general condition improved and patient started to complain of painful hips and shoulders. On further inquiry, patient gave a history of using 15 mg prednisolone daily for aplastic anemia for two years. No supplement or antiresorptive therapy was given to him. Clinical examination showed that both the shoulder joints were dislocated and any attempt to move the hips and right wrist caused tremendous amount of pain. Radiographs showed that bilateral anterior dislocation of shoulder with bilateral fracture surgical neck, Smith's fracture of the right radius and bilateral fracture of the neck of femur (Garden IV) (Figures and ). Under general anesthesia both shoulder joints were reduced, fracture necks of femur were fixed with two cannulated screws (), Smith's fracture was reduced and plaster of paris application was done. Two weeks later, bone mineral density of the spine (Dual Energy X-ray Absorptiometry, DEXA scan) showed severe osteoporosis with T score of −2.9. Last follow-up appointment was four years from the incidence and revealed no pain but rather painless limping. Examination showed right hip and left hip had limited range of movements and pain at extreme degree. The range of movements of the shoulder joints were normal. |
A 42-year-old man with EDS type IV was admitted to our department with a rapidly progressive cervical pulsatile and painful swelling on the right side (). Apart from the swelling, a neurological examination also showed right-sided palsy of the hypoglossal nerve. The patient's history indicated that, in 1999, direct revascularization using a vessel graft to treat an extracranial giant aneurysm of the left ICA had failed due to technical problems in performing an adequate anastomosis causing left hemispheric ischemia. Neurological impairment such as right-sided hemiparesis or aphasia was not seen at the current presentation.\nCerebral digital subtraction angiography (DSA) showed a giant aneurysm of the right ICA (C1 segment) measuring 10×7×7 cm, extending from the carotid bifurcation to the base of the skull (). A largely dilated basal artery and left posterior communicating branch indicating compensatory supply to the left hemisphere was also demonstrated. A Doppler-controlled balloon occlusion test of the right ICA revealed a moderate insufficiency of the middle cerebral artery (MCA) territory and additional collateralization of the right hemisphere via the ophthalmic branches. MRI demonstrated ischemic lesions of the left hemisphere as a result of the previous occlusion of the left ICA.\nThe radiological findings were then thoroughly discussed in an interdisciplinary conference including neuroradiological, neurological and neurosurgical colleagues in order to achieve a low-risk and adequate treatment of the growing aneurysm of the right ICA. The agreed strategy consisted of securing right cerebral perfusion via flow redirection through a double-barrel extracranial–intracranial bypass followed by surgical trapping of the aneurysm. The double-barrel bypass was successfully placed from the superficial temporal artery to two M4 cortical branches of the right MCA (). Both branches showed sufficient flow in postoperative angiographic studies. Prior to definite occlusion, a temporary ICA occlusion test was conducted using an eclipse balloon in order to confirm sufficient contrast perfusion of the right hemisphere through the previously placed tandem bypass (). The occlusion test showed good flow through the bypass and additional ophthalmic collateral filling, so we were able to proceed with the permanent elimination of the aneurysm from the circulation. Surgical trapping of the aneurysm failed due to the risk of sacrificing the external carotid artery. A decision was therefore made to occlude the aneurysm by means of endovascular coiling. Two separate sessions were needed to occlude both the distal and proximal segments of the giant aneurysm. The distal segment was successfully occluded with platinum spiral coils (). A stent was then placed in the right external carotid artery to secure its patency and, from there, stent-supported coiling was performed to occlude the proximal segment (). Secure proximal placement of the coils was not possible in a single-catheter technique owing to the high flow turbulence within the aneurysm sac. By using a double-catheter method, two coils could be placed simultaneously directly outside the stent wall facing the proximal segment of the extracranial ICA. Immediately after the procedure the patient had a transient ischemic attack with latent left-sided hemiparesis which regressed within 48 h. Ischemic lesions were excluded in post-procedural non-enhancement CT scans. Minor surgery was later performed to drain the residual hematoma in the aneurysm sac. The bulging skin collapsed instantly, reducing the large visible swelling on the patient's neck. The patient recovered rapidly from the individual procedures without any additional impairment and was discharged in an unaltered clinical/neurological condition.\nFollow-up at 2 months revealed a good clinical result without any deficits with slight recurrence of the swelling on the right side of the neck. CT and CT angiography showed a hypodense non-enhancing filling of the remaining aneurysm sac. Density measurements indicated the filling to be serous fluid which was safely drained via CT-assisted puncture. Again the swelling collapsed immediately, leaving the patient with a good cosmetic result (). The patient died a year later due to a ruptured aneurysm of the splenic artery. |
A 45-year-old woman presented to our hospital with multiple lung nodules. She had a history of poorly differentiated thyroid carcinoma, diagnosed 7 months prior to admission, at an outside hospital. The patient was healthy otherwise and reported no radiation exposure or any family history of thyroid cancer. The initial work-up at the time of discovery of the right thyroid nodule included fine needle aspiration and core biopsy, with findings consistent with poorly differentiated thyroid carcinoma. The patient then underwent a total thyroidectomy and central neck lymph node dissection. The pathologic diagnosis from the outside hospital reported a 2.8 × 2.4 × 1.1 cm tumor in the right thyroid without extrathyroidal extension or lymph node metastasis. However, both capsular invasion and extensive vascular space invasion were noted. Based on the tumor size, tumor extension and lymph node status, the tumor was designated as Stage II (pT2 pN0 pMx). IHC staining showed that the tumor cells were positive for thyroglobulin and thyroid transcription factor 1 (TTF1). An immunostain for p53 was also performed at the outside hospital and showed a small focus (< 1 cm) with p53 positivity, suggesting a diagnosis of anaplastic thyroid carcinoma.\nAt our institution, the diagnosis was revised, based on review of both the primary thyroid tumor and the current lung metastases. Both tumors were remarkable for biphasic malignant components: the carcinoma and the sarcoma. The carcinoma component showed a poorly differentiated microfollicular type thyroid carcinoma, composed of sheets and islands of tightly packed thyroid follicles with dense colloid. The tumor nuclei were small and round with vesicular chromatin, resembling those of typical poorly differentiated follicular thyroid carcinoma. Admixed with the epithelial component were malignant spindle cells with small round blue cell type morphology. Focally, rhabdomyosarcoma-like cells with eosinophilic cytoplasm were appreciated. No heterologous cartilage or bone components were identified. The IHC staining performed at the outside hospital showed that the thyroid carcinoma (epithelial) component was positive for thyroglobulin, PAX8 and TTF1 (Fig. ). The sarcoma (spindled) component was negative for all thyroid carcinoma markers (TTF-1, thyroglobulin and PAX8), but was positive for vimentin and focally positive for myogenin (supporting skeletal muscle differentiation) consistent with mesenchymal differentiation. Interestingly, the foci of vascular space invasion contained both epithelial and mesenchymal components as well.\nThe patient received Taxol with Carboplatin for 7 weeks followed by radiation therapy. Her thyroglobulin level rose from 1.2 ng/mL to 25.40 ng/mL 5 months after completion of the chemo-radiation therapy, suggesting progression of the disease. A follow-up CT scan of the chest showed multiple newly developed nodules (ranging from 1 to 2 cm) in the right lung, highly suspicious for metastases. The patient underwent a right thoracotomy, right lung resection/metastasectomy. The surgery was uneventful with negative resection margins. However, the patient’s general condition deteriorated and she succumbed to the disease 4 months later.\nHistological examination of the lung nodules revealed similar tumor morphology and tumor differentiation when compared to the original thyroid tumor, which is somewhat unusual for a biphasic carcinosarcoma (Fig. ). Tumor necrosis was also present. Mutational analysis using a next-generation sequencing based assay showed that the neoplastic cells from the lung metastasis were devoid of genomic alterations for known thyroid cancers, including BRAF, RAS family (KRAS, NRAS and HRAS), EGFR, PTEN, TERT, PI3Kinase or RET. BRAF or RAS family are known as the most commonly altered genes in papillary thyroid cancers. Other molecular mutations reported in the development of anaplastic thyroid carcinoma include p53, PAX8/PPAR gamma rearrangement []. None of the mentioned gene mutations were identified in our patient.\nHowever, an interesting finding in this case is the presence of a point mutation in DICER1 (E1705K) that has previously been associated with differentiated thyroid carcinoma [, ]. Whether the DICER1 (E1705K) mutation is the underlying genetic event leading to the initiation of tumorigenesis or is downstream to other gene alterations in tumor development is largely unknown. Additional mutations of unknown significance were also detected in this tumor including FLCN (R239H), POLD1 (Q684H) and SYK (R217L). These variants have not been adequately characterized in the scientific literature and their prognostic and therapeutic significance is unclear. |
A 24-year-old male patient was referred to our ER as a case of mediastinal tumor compressing the airways. On arrival, the patient was in severe respiratory distress; he had stridor, tachypnea, and marked desaturation (Pao2 less than 60%). The patient was not accompanied by any family member; thus, obtaining history was not possible at that point. The chest x-ray done at the referring hospital showed a huge right paratracheal mediastinal mass compressing the airways from the right side (). The patient was taken to the OR for intubation under fiberoptic bronchoscopy guidance. The fiberoptic bronchoscopic evaluation showed significant compression of the trachea from the right side with no endobronchial lesions. A small ETT (size 5) was successfully inserted. The patient was then taken to the radiology department for a chest CT with IV contrast. Chest CT showed a huge vascular mass (15 × 20 cm) consisting of a central pool of contrast with surrounding compressed tissue. The mass markedly compressed the trachea and the right mainstem bronchus with marked shifting of the mediastinum to the left side (). The SVC was markedly compressed, thinned out, and shifted by the pseudoaneurysm to the right side. The study showed the proximal 2 cm of the innominate artery, followed by the pseudoaneurysm. The right common carotid can be seen at the upper end of the pseudoaneurysm. There was an interruption of the right subclavian artery at its origin (Figs. ). Collaterals were seen supplying the right upper limb.\nThe patient was taken immediately to the OR, and the mediastinum was explored through a midline sternotomy. A huge saccular aneurysm was identified arising from the lateral wall of the innominate artery with a fibrous capsule firmly adherent to the surrounding structures. The aneurysm was dissected from the surroundings, resected, and the innominate artery's lateral wall was repaired at the origin of the right common carotid artery. The distal end of the subclavian artery was arising from the far end of the pseudoaneurysm. We ligated the distal end of the right subclavian artery. Postoperatively, history was taken from the patient. He had a penetrating chest injury by a sheep horn in the right infraclavicular region 15 years prior to presentation. The patient's postoperative course was uneventful, and his respiratory symptoms resolved. The right upper limb was properly functioning, and CT angiography showed excellent filling of the right subclavian artery from the collateral circulation. Pseudoaneurysm of the innominate artery should be suspected as a rare complication in chest trauma. |
A 60-year-old Japanese woman with a history of rheumatoid arthritis and NF1 was admitted to our hospital for the treatment of a tumor of a pancreas. She had no symptoms, but an abdominal ultrasonography screening examination had revealed a hypoechoic mass in the head of a pancreas. Laboratory data, including measurement of tumor markers, were within the normal ranges, and her insulin and glucagon levels were also within the normal ranges. However, the plasma gastrin level was elevated at 580 pg/mL (30 to 150 pg/mL). A computed tomography (CT) examination revealed a hypervascular tumor measuring 14 mm in diameter in the pancreas head (Figure ). Magnetic resonance imaging (MRI) also revealed a massive tumor in the head of the pancreas. A duodenal endoscopy revealed that the lumen of the patient's duodenum was not compressed by the extraluminal tumor, and findings on endoscopic retrograde cholangiopancreatography showed that her main pancreatic duct was not stenosed or blocked. We diagnosed the patient as having a pancreatic neuroendocrine tumor and planned to perform a tumoral enucleation from her pancreas.\nA whitish elastic hard nodule was easily removed from the pancreatic parenchyma, but the tumor was connected to her duodenal wall via a stalk. Consequently, we performed a tumor resection with a duodenal wedge resection. As a small whitish nodule was also found in the patient's jejunum during the initial operation, a segmental jejunectomy was also performed. A histological examination of frozen sections of the patient's tumors revealed spindle cells with connective tissues. Thus, we diagnosed the patient as having multiple GISTs and did not perform a more radical resection.\nMacroscopically, the resected specimens consisted of solid and hard masses that were connected to the patient's duodenal and jejunal walls but not to the parenchyma of the head of her pancreas. Microscopically, this neoplasm originated from the muscularis propria of the duodenum wall and consisted of spindle cells in a trabecular pattern without necrosis (Figure ). Less than five mitoses per 50 high-power fields were observed.\nOn immunohistochemistry, both of the patient's tumors were diffusely positive for the type III receptor tyrosine kinase (TK) named KIT (Dako Cytomation, Copenhagen, Denmark; Figure ), whereas her duodenal tumor was focally positive and her jejunal tumor was diffusely positive for CD34 (Nichirei, Tokyo, Japan). Both of the patient's tumors were negative for smooth muscle actin (Dako Cytomation, Copenhagen, Denmark) and S-100 (Nichirei, Tokyo, Japan). We finally diagnosed the patient as having duodenal and jejunal GISTs of uncommitted type. Patients with these GISTs are regarded as being very low risk of recurrence according to the National Comprehensive Cancer Network (NCCN) guidelines []. The patient has remained healthy for two years without any recurrences after surgery. |
The patient is a 74-year-old male with comorbidities of coronary artery disease, congestive heart failure, hypertension, and renal insufficiency who presented to an outside hospital with back pain and left upper extremity weakness associated with high fevers and urinary incontinence. Outside spinal imaging showed a large spinal abscess; therefore, he was transferred to our institution for a higher level of care. On presentation, the patient was in septic shock requiring fluid boluses and inotropic agents to stabilize him. Further history was obtained from the family as follows:On November 2016, he underwent an elective right TKA complicated by an early MRSA PJI associated with bacteremia On May 0f 2017, he underwent resection of the hardware in the knee, incision and drainage, followed by 12 weeks of daptomycin therapy Five months later, on October of 2017, due to relapse, he had a second debridement of the right knee for source control as well as left ankle incision and debridement followed by another 8 weeks of daptomycin for this relapse of infection A daptomycin-susceptible, vancomycin-susceptible MRSA was isolated from blood and both surgical sites, knee and ankle, on both occasions He had ongoing thoracic back pain since 2016 which was monitored radiographically by his local providers, until the development of spinal epidural abscess with upper extremity weakness, which prompted his current hospitalization in January of 2018\nReview of systems on presenting to our institution was significant for general weakness and malaise, right shoulder and thoracic back pain, and constipation from narcotics. He was hemodynamically unstable requiring inotropic support. He was awake and oriented, following commands with intact speech. There were no cranial nerve deficits. On motor testing, he had normal muscle bulk with generalized hypotonia. There was no movement of his left upper extremity. He had 2/5 strength on his right upper extremity and 2/5 strength on his bilateral lower extremity. There was decreased sensation to light touch on his left side. Reflexes were globally decreased with negative Hoffman and Babinski signs. The white blood cell count was 30,000/L, and procalcitonin was 4.88 ng/ml. Blood cultures grew MRSA rapidly. Repeat imaging of the brain and spine at our institution showed extensive epidural phlegmon throughout the cervical, thoracic, and lumbar spine with intracranial expansion into the posterior fossa beneath the cerebellum with pockets of possible early organizing abscess within the phlegmon (). Brain imaging identified no discrete abscess or leptomeningeal enhancement.\nNeurosurgery immediately evaluated the patient and promptly performed a cervical spine decompression of C1–C7 and thoracic spine decompression of T5–T7. Operatively, a large epidural abscess was found, drained, and washed out. He was started on vancomycin every 12 hours with trough vancomycin levels being therapeutic. The patient subsequently underwent irrigation and debridement of the right knee, left ankle, and left great toe as well at our institution; all surgical sites grew MRSA with vancomycin MIC of 1 mcg/ml. Despite attempts at source control and optimal pharmacokinetic dosing of vancomycin with a trough level of 20.5 mcg/ml on day 5, he had refractory MRSA bacteremia. Infectious disease deemed he had failed daptomycin therapy; therefore, ceftaroline 600 mg every 8 hours (MIC of 0.38 mcg/ml) was added to vancomycin. Repeat blood cultures showed clearance of bacteremia after 48 hours of initiation of the combination therapy. His left ankle and right knee continued to yield MRSA. Due to his multiple comorbidities and need for more aggressive source control of his infection, i.e., amputation of the leg, palliative care was sought by the family, and he died a few days later. |
A 54-year-old man diagnosed with locally advanced central squamous cell carcinoma was hospitalized in our hospital. The patient had cough and shortness of breath for 3 months. Chest CT showed central lung cancer in the upper lobe of the right lung, with tumor invading the trunk of the right pulmonary artery, the main bronchus and the upper lobe bronchus (Fig. ). Squamous cell carcinoma was confirmed by further bronchoscopy. Three courses of neoadjuvant chemotherapy were performed before surgery. The patient refused to continue chemotherapy and asked for active surgical treatment. According to the TNM staging system, the clinical stage of this tumor is cT4N1M0.\nGeneral anesthesia was induced and maintained according to standard protocols during surgical preparation. Then right pneumonectomy was performed, and the surgical incision was located at the posterolateral side of the fourth intercostal space. The pulmonary artery, pulmonary vein and bronchus were cut off with a stapler, with intraoperative bleeding of about 100 ml. After the operation, the thoracic drainage tube was clamped, and the patient returned to the ward after waking up. Three hours after the operation was completed, the patient suddenly lost consciousness while sitting in bed chatting with his wife. The patient’s blood pressure was low (76/53 mmHg), and the indexes of heart rate, respiration and oxygen saturation were normal. After opening the thoracic drainage tube, a small amount of tension gas and 200 ml of bloody fluid was discharged. We immediately performed CT examination and excluded brain lesions. CT examination showed that there was a large amount of gas in the tissue space between the chest and neck (Fig. ). Blood clots accumulated in the thoracic cavity, and CT angiography showed no abnormalities in the major cerebral arteries (Fig. ). However, multiple free air can be seen in the blood vessels of bilateral frontal sulcus (Fig. ). In addition to free air, suspicious cerebral infarction was also seen in the right occipital lobe (Fig. ). It is presumed that air entering the cerebral circulation led to air embolism. The patient had seizures soon after the CT scan, manifesting as binocular gaze and tremor of limbs. Then the patient was quickly transferred to ICU. Respiratory assistance, mannitol dehydration and empiric antibiotic treatment were used after ICU transfer. 800 ml pleural fluid was drained from the thoracic cavity 6 h after the operation, and a second thoracotomy was performed to stop bleeding. Then blood clot was removed from the thoracic cavity. After the operation, the patient was sent back to the ICU for ventilator-assisted breathing, and the head was protected by mild hypothermia using an ice blanket to prevent excessive brain damage. At the same time, mannitol dehydration was used to reduce brain edema and anti-infection treatment was carried out. On the next day, the brain CT showed a significant decrease of air in the brain (Fig. ). There was no significant increase in cerebral infarction lesions compared with the first day. After 3 days of ventilator-assisted breathing, the patient’s condition gradually improved. On the third day after air embolism, the brain CT was reexamined. There were patchy low density areas in bilateral thalamic basal ganglia, temporal lobe and occipital lobe, which was cerebral infarction lesions. The patient was discharged after 25 days of treatment after cerebral infarction. He was conscious at the time of discharge, with neurological impairment symptoms of bilateral in which symptoms of left limb are more serious. The process from onset to recovery was smooth. After 3 months of follow-up, most of the neurological deficit symptoms had been recovered except for the left upper limb (Fig. ). |
A 57-year-old male electronic engineer presented with severe burning dysesthesia and pain in D4-12 distribution on the right side of his chest and abdomen. He also experienced troublesome allodynia leading to decreased sleep, excessive day time sleepiness and fatigue. The symptoms according to him started 6 months prior to his present visit after he had a chest tube insertion for drainage of pleural effusion. On the basis of pleural fluid analysis he was subsequently diagnosed to have pulmonary tuberculosis and was started on antituberculous treatment.\nHe had elevated blood sugar at that time with fasting blood sugar (FBS) 246 mg/dl and postprandial blood sugar (PPBS) 360 mg/dl with glycated hemoglobin (HbA1c) 9.2%. He was first diagnosed to have diabetes 3 years ago and was on irregular treatment for the same. He was initiated on insulin therapy and discharged. A week later he noticed a right abdominal bulge especially after taking a heavy meal or while trying to get up from a lying posture. This was associated with a burning pain over the right lower chest and upper abdomen. The protrusion gradually increased in size.\nHe was extensively investigated for his “painful abdominal mass” including a gastrointestinal scopy and ultrasonography (USG) abdomen, however no abnormality could be detected. Meanwhile his blood sugar remained uncontrolled, he stopped insulin and changed to herbal medications. On initial evaluation at our hospital, his HbA1c was 8.9%. He was initially investigated by our gastroenterology department for his painful abdominal swelling. Since all results were within normal limits he was referred to neurology department for further evaluation. Meanwhile he also developed a right proximal lower limb weakness associated with wasting of thigh muscles leading to buckling at right knee joint. He had no relief of his painful abdominal sensory symptoms.\nOn examination there was a healed scar at the site of intercostal drain insertion in right fifth intercostal space on the anterior axillary line. All peripheral pulses were felt. Right thigh girth was 2 cm less compared to left side. He had weakness of hip adduction, knee extension, and hip extension all on the right side. The right abdominal muscles were weak with a protrusion of the abdomen mimicking a mass []. Abdominal reflexes were absent in all quadrants. Right knee jerk was absent and sensation to crude touch was diminished by 20% on the right side extending from D4 dermatome to right knee.\nOn investigations, he continued to have high blood sugars on admission. FBS 423 mg/dl and PPBS 384 mg/dl with HbA1c 8.9%. Cerebrospinal fluid (CSF) revealed normal sugar with protein 79 mg/dl and 2 cells/mm3, mononuclear. Magnetic resonance imaging (MRI) cervical, dorsal, and lumbosacral spine with contrast showed mild cervical canal stenosis at C3-C6. No evidence of spinal arachnoiditis. Nerve conduction velocity study (NCV); right femoral compound muscle action potential (CMAP) amplitudes were reduced and paraspinal electromyography studies (EMG) revealed denervation potential from thoracic and lumbar segments. Toxicological screening for herbal medication revealed no heavy metals. USG abdomen was normal. |
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